Acute Rupture of Primary Hepatic Angiosarcoma with Kasabach-Merritt Syndrome: A Rare Case Report and Literature Review

Acute Rupture of Primary Hepatic Angiosarcoma with Kasabach-Merritt Syndrome: A Rare Case Report and Literature Review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Acute Rupture of Primary Hepatic Angiosarcoma with Kasabach-Merritt Syndrome: A Rare Case Report and Literature Review Lin Ye, Wanrong Yue, Hao Shi, Renjian Li, Jun Weng, Haozhe Zhou, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6461668/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 22 Oct, 2025 Read the published version in World Journal of Surgical Oncology → Version 1 posted 13 You are reading this latest preprint version Abstract Background Primary hepatic angiosarcoma (PHA) is an extremely rare malignant tumor originating from vascular endothelial cells, accounting for only 0.1-2% of primary liver malignancies. Kasabach-Merritt syndrome (KMS) is a rare complication characterized by thrombocytopenia, coagulation dysfunction, and microangiopathic hemolytic anemia, seldom reported in adult patients with hepatic angiosarcoma. Case presentation We describe a 74-year-old female with a history of vinyl chloride exposure who presented with acute right upper quadrant pain, shock, and scattered purpura. Laboratory tests revealed thrombocytopenia and coagulation abnormalities. Imaging demonstrated a large ruptured hepatic mass with hemoperitoneum. The patient underwent emergency right hemihepatectomy, with pathology confirming hepatic angiosarcoma. Postoperatively, the patient's coagulation parameters normalized, confirming resolution of KMS. The patient showed no recurrence at 9-month follow-up. Conclusion Acute rupture of hepatic angiosarcoma with KMS is a life-threatening condition requiring rapid multidisciplinary intervention. Emergency hepatectomy not only controlled the hemorrhage but also removed the lesion causing KMS. Clinicians should maintain a high index of suspicion for hepatic angiosarcoma in patients presenting with spontaneous hepatic hemorrhage and coagulation abnormalities. Hepatic angiosarcoma Kasabach-Merritt syndrome Acute rupture Emergency hepatectomy Coagulopathy Figures Figure 1 Figure 2 Figure 3 Introduction Primary hepatic angiosarcoma(PHA) is an extremely rare malignant tumor originating from endothelial cells, blood vessels, and lymphatic vessels, accounting for only 0.1-2% of all primary hepatic malignancies and 4% of all angiosarcomas. This tumor exhibits highly aggressive characteristics and is typically discovered at an advanced stage with multifocal lesions, resulting in a poor prognosis with a median survival of approximately 6 months [ 1 , 2 ]. Although the etiology remains incompletely understood, studies have indicated that exposure to vinyl chloride, thorium dioxide, arsenic, and long-term use of androgens may be associated with its development [ 3 , 4 ]. Kasabach-Merritt syndrome (KMS) was initially described in infant hemangiomas and is characterized by consumptive coagulopathy, manifesting as thrombocytopenia, hypofibrinogenemia, and microangiopathic hemolytic anemia[ 5 ]. While KMS is commonly seen in infantile kaposiform hemangioendothelioma or tufted hemangioma, its occurrence in adult hepatic angiosarcoma is extremely rare[ 6 ]. Spontaneous rupture of hepatic angiosarcoma is a life-threatening complication with an incidence of approximately 10–25%[ 7 ]. The mechanism of rupture may be related to the abnormal vascular structure of the tumor, its invasive growth pattern, and infiltration of adjacent blood vessels. When spontaneous rupture is accompanied by KMS, the clinical situation becomes even more critical, requiring prompt diagnosis and management[ 8 ]. In the published literature to date, cases of adult hepatic angiosarcoma with KMS and acute rupture reported are extremely rare. This paper reports a case of a 74-year-old female with acute rupture of hepatic angiosarcoma accompanied by KMS who was successfully treated with emergency right hemihepatectomy. Case Presentation A 74-year-old female with a 5-year history of well-controlled hypertension presented to our emergency department with "sudden onset of severe right upper abdominal pain accompanied by nausea and vomiting for 3 hours." Two months prior, she had sought medical attention for fatigue and was found to have mild anemia (hemoglobin 103 g/L) and thrombocytopenia (platelet count 71×10^9/L). At that time, she was diagnosed with "anemia" and prescribed oral iron supplements without further follow-up. Clinical details of the patient's diagnostic and treatment process are illustrated in Fig. 1 . Upon admission, the patient appeared pale and in poor general condition. Vital signs showed: blood pressure 86/45 mmHg, heart rate 125 beats/min, respiratory rate 22 breaths/min, and oxygen saturation 92% (with supplemental oxygen). Physical examination revealed scattered purpura on the abdomen and lower extremities (Fig. 2 A). Abdominal examination showed significant right upper quadrant tenderness with rebound pain and muscle guarding, enlarged hepatic dullness, and positive shifting dullness. Laboratory tests indicated: white blood cell count 15.2×10 9 /L, hemoglobin 76 g/L, platelet count 45×10 9 /L; prothrombin time 18.5 seconds, activated partial thromboplastin time 45.7 seconds, fibrinogen 1.2 g/L, D-dimer 3.5 mg/L; alanine aminotransferase 87 U/L, aspartate aminotransferase 156 U/L, total bilirubin 28 µmol/L, direct bilirubin 13 µmol/L, and albumin 29 g/L. Bedside ultrasound examination revealed large amounts of free fluid in Morrison's pouch (hepatorenal recess) and throughout the peritoneal cavity, consistent with hemoperitoneum(Fig. 2 B). Emergency contrast-enhanced abdominal CT revealed a low-density mass measuring approximately 10.0 cm × 16.0 cm in the posterior segment of the right liver lobe, with heterogeneous enhancement in the arterial phase and progressive filling in the portal venous and delayed phases. Fluid collection with a density of approximately 45–50 HU was observed around the mass, suggestive of blood. Massive intraperitoneal fluid was also noted (Fig. 2 C- 2 F). Given the clinical presentation of suspected hepatic tumor rupture leading to hypovolemic shock with coagulation dysfunction, immediate multidisciplinary assessment was performed. Initial resuscitation included intravenous fluids, blood product transfusions, and vasopressor support, followed by emergency exploratory laparotomy. Intraoperatively, approximately 3000 mL of bloody fluid and clots were found in the peritoneal cavity. A dark red mass measuring approximately 10 cm × 9 cm was identified in the posterior segment of the right liver lobe, with visible rupture and active bleeding points on its surface. Based on these findings, a right hemihepatectomy was performed. Intraoperative blood loss was approximately 2500 mL, necessitating transfusion of 8 units of packed red blood cells and 800 mL of fresh frozen plasma. Pathological examination of the gross specimen (right liver lobe tissue measuring 16 cm × 11 cm × 8 cm) revealed a dark red mass measuring 8.5 cm × 7.5 cm with indistinct borders. Portions of the mass had a sponge-like appearance with areas of necrosis and hemorrhage (Fig. 3 A& 3 B). Microscopically, the tumor consisted of atypical spindle-shaped cells arranged in bundles or irregular sheets, forming irregular vascular spaces lined by atypical endothelial cells. The tumor cells exhibited large, hyperchromatic nuclei, scant cytoplasm, and frequent mitotic figures (7–8 per 10 high-power fields) (Fig. 3 C). Immunohistochemical studies showed: CD31(+), CD34(+), ERG(+), FLI-1(+), Ki-67 (approximately 25%+), CK(-), Hepatocyte(-), and GPC-3(-).(Fig. 3 D- 3 G). The pathological diagnosis was hepatic angiosarcoma. Discussion Primary Hepatic angiosarcoma(PHA) is an extremely rare malignant tumor originating from malignant proliferation of hepatic endothelial cells, characterized by high invasiveness and widespread distribution. Despite its low incidence, PHA is the most common primary mesenchymal malignancy of the liver in adults, accounting for less than 2% of all primary hepatic malignancies[ 9 ]. Epidemiological studies show that this disease predominantly affects males over 60 years of age, with a male-to-female ratio of 2–4:1. Regarding the pathogenesis of PHA, current research has found that approximately 25% of cases are associated with environmental and occupational exposure factors, including vinyl chloride monomer, arsenic compounds, thorium dioxide (Thorotrast) contrast agents, synthetic steroids, as well as systemic diseases such as hemochromatosis and neurofibromatosis; while the etiology of the remaining 75% of cases remains unclear, possibly involving complex molecular pathological mechanisms. long-term exposure to vinyl chloride monomer and arsenic compounds is considered a significant risk factor for the development of hepatic angiosarcoma, which has been confirmed in epidemiological investigations of populations around industrial areas[ 10 – 12 ]. In this case, through detailed history taking, we discovered that the patient had worked in a plastic products factory for over 30 years, primarily responsible for processing and handling polyvinyl chloride products, with long-term exposure to a vinyl chloride monomer environment. This known causative factor for hepatic angiosarcoma likely played a significant role in the development of the disease in this patient. The interval from the patient's initial exposure to these carcinogens until the appearance of the tumor spanned several decades, consistent with the 10–40 year latency period following vinyl chloride exposure reported in previous literature. This case once again emphasizes the importance of occupational and environmental exposure history in the diagnosis of unexplained vascular tumors of the liver, while suggesting that we should strengthen health monitoring and screening for populations with similar exposure histories. The clinical presentation of hepatic angiosarcoma is diverse and lacks specificity, which constitutes the primary reason for diagnostic difficulty and delay. According to the literature, abdominal pain is the most common chief complaint, with approximately 60% of patients initially presenting with abdominal pain as their primary symptom[ 13 ]. This is followed by fatigue, weight loss, abdominal distension, anorexia, and fever—nonspecific symptoms similar to those of other chronic liver diseases. Physical examination may reveal hepatomegaly, ascites, or jaundice, with approximately one-third of patients demonstrating obvious hepatic masses on initial diagnosis. Laboratory tests typically show abnormal liver function, but without specific changes; hepatitis B immunological testing is usually negative, and currently there are no reliable serological markers for early diagnosis. As the disease progresses, patients may develop serious complications such as cirrhosis and liver failure; due to the vascular nature of the tumor, approximately 17%-27% of patients experience spontaneous tumor rupture with hemoperitoneum, a life-threatening emergency typically presenting as sudden abdominal pain and hemorrhagic shock[ 14 ]. Additionally, some patients may develop KMS, characterized by thrombocytopenia, decreased fibrinogen, and consumptive coagulopathy. Hepatic angiosarcoma is highly malignant, with approximately 25%-40% of patients already exhibiting hematogenous metastases to organs such as the lungs, spleen, or bones at the time of diagnosis[ 15 ]. In this case, the patient initially presented with acute abdominal pain and shock, accompanied by laboratory findings indicating coagulation dysfunction and thrombocytopenia, and imaging revealing hepatic tumor rupture—a combination of clinical presentations suggesting the possibility of hepatic angiosarcoma. KMS, also known as Kasabach-Merritt phenomenon (KMP), is a rare complication of vascular tumors, initially described primarily in children with kaposiform hemangioendothelioma or tufted angioma, but has recently been reported in adult cases of hepatic angiosarcoma as well(Table 1 )[ 16 ]. Research indicates that tumor size is one of the independent risk factors for the development of KMS, with truncal lesions more likely to develop KMS than non-truncal locations[ 17 ]. Ji et al. confirmed that approximately 70% of patients with kaposiform hemangioendothelioma presented with KMS, and that age at tumor discovery, morphology, and tumor size were independent risk factors for KMS development[ 18 ]. Table 1 Summary of Reported Cases of Hepatic Angiosarcoma with Kasabach-Merritt Syndrome. Case Age (years) Sex Symptoms Viral Hepatitis Chemical Exposure Platelet (10 9 /L) FDP (µg/mL) Fibrinogen (mg/dL) Treatment Survival Time Cause of Death Wadhwa et al. [5] 44 Male abdominal pain and jaundice NS Negative 57 NS 60 liver needle biopsy NS NS Fujii et al. [8] 76 Female Gingival bleeding Negative NS 56 233 124 Supportive care 4 months Abdominal bleeding liver failure Deng et al. [16] 59 Male Hemoptysis nodules in lungs Negative NS 90 56.22 87 Partial hepatectomy Paclitaxel adjuvant chemotherapy (3 cycles) 9 months Tumor metastasis Zhang et al. [19] 56 Male Disturbance of consciousness, jaundice NS NS 21 74.7 81.6 Liver transplantation 2 months Tumor metastasis Tang et al. [20] 66 Female Right upper abdominal pain multiple skin purpuras Negative NS 37 110.9 150 TACE 6 months Multiple liver metastases González et al. [22] 70 Male Right hypochondrium pain and mass sensation Negative NS 42 NS NS Right hepatectomy NS NS Cui et al. [23] 70 Male Chest tightness、Dyspnea abdominal discomfort Positive NS 95 127.6 154 Supportive care, attempted interventional radiology 2 weeks Pulmonary hemorrhage Tezuka et al. [25] Newborn Female Tachypnea、Cyanosis Hypotension abdominal vascular murmur NS NS Normal 22.6 71 Corticosteroids, coil embolization > 9 months N/A Our case 74 Female right upper abdominal pain multiple cutaneous purpuras Negative Vinyl chloride exposure 71 250 120 Right hepatectomy > 9 months N/A (To date, No recurrance ) NS = Not Specified; N/A = Not Applicable; FDP = Fibrin Degradation Products; TACE = Transarterial Chemoembolization. The pathophysiology of KMS remains incompletely elucidated, but is generally believed to be associated with tumor endothelial cells inducing platelet activation and aggregation, while local turbulence and abnormal vascular structures within the tumor may promote the coagulation cascade. Platelet sequestration and consumption within the tumor leads to thrombocytopenia; red blood cells are destroyed in irregular vascular spaces, resulting in microangiopathic hemolytic anemia; simultaneously, continuous coagulation activation depletes coagulation factors, causing hypofibrinogenemia and coagulation dysfunction. Notably, while liver failure is commonly caused by drug toxicity and viral hepatitis, liver failure induced by hepatic angiosarcoma is relatively rare[ 19 , 20 ]. In the present case, the patient had experienced fatigue accompanied by anemia and thrombocytopenia two months prior to admission, suggesting that the tumor and KMS may have been present for some time. Based on the patient's coagulation abnormalities and thrombocytopenia upon admission, combined with clinical symptoms, we diagnosed KMS. Regarding the radiological diagnosis of hepatic angiosarcoma, typical features include heterogeneous enhancement in the arterial phase with progressive filling in the portal venous and delayed phases, but these features have limited specificity. The hepatic mass shown on the emergency CT scan of this patient corresponded with these characteristics, but due to the emergency situation, more detailed examinations could not be completed, and a definitive diagnosis of hepatic angiosarcoma could not be established preoperatively. The final diagnosis of hepatic angiosarcoma with KMS was confirmed through surgery and pathological examination. The gradual normalization of the patient's coagulation function and platelet count postoperatively further confirmed the causal relationship between these two conditions. Therapeutic options for hepatic angiosarcoma are limited, with surgical resection currently remaining the only potentially curative method. However, due to the disease's invasive nature, most patients are diagnosed at an advanced stage, with research indicating that only approximately 20% of patients are suitable for surgical intervention. The invasive characteristics and multifocality of angiosarcoma further restrict surgical applications, with partial hepatectomy typically considered only when the lesion is confined to a single hepatic lobe. Nevertheless, surgical resection significantly improves patient prognosis[ 21 , 22 ]. Wilson et al.'s research found that patients undergoing surgical resection had a median survival of 33.4 months, significantly superior to non-surgical patients[ 23 ]. Martínez et al.'s retrospective study similarly demonstrated that surgically treated patients had a median survival of 8 months, compared to merely 2 months for those without surgical intervention[ 24 ]. For the patient in this case, emergency surgical treatment is both reasonable and necessary due to tumor rupture accompanied by hemodynamic instability. In critical situations of tumor rupture with intra-abdominal hemorrhage, transarterial embolization (TAE) is the preferred method for controlling life-threatening bleeding, though it carries risks of rebleeding and poor prognosis[ 25 – 27 ]. Liver transplantation was previously considered a treatment option, but due to high post-operative recurrence rates and distant metastasis risks, it is currently rarely recommended. However, individual successful cases merit attention, such as the report by Dhaliwal et al. of a 61-year-old female patient who showed no tumor recurrence 16 months after liver transplantation, suggesting that transplantation might offer a treatment opportunity for select patients under specific circumstances[ 28 ]. For unresectable cases, treatment options are even more limited. Studies indicate that hepatic angiosarcoma demonstrates poor responsiveness to radiation therapy, exhibiting significant radioresistance[ 30 ]. Regarding chemotherapy, drugs such as docetaxel, paclitaxel, and doxorubicin have shown certain efficacy, particularly in advanced-stage patients[ 29 ]. Systematic studies reveal that patients receiving chemotherapy have longer survival times and lower mortality risks compared to those receiving no chemotherapy, though the overall effect remains unsatisfactory, indicating that current treatment strategies still require improvement[ 30 , 31 ]. In recent years, molecular targeted therapy and immunotherapy have brought new hope for hepatic angiosarcoma patients. Gera et al. discovered BRAF mutations in some hepatic angiosarcoma patients, with those patients demonstrating excellent responses to the BRAF inhibitor vemurafenib. Additionally, pazopanib, PD-1 inhibitors, and therapies targeting vascular endothelial growth factor (VEGF) have shown research progress[ 32 ]. Lin et al. reported successful cases of transarterial chemoembolization (TACE) combined with targeted immunotherapy[ 25 ]. Even more encouragingly, recent studies have explored combination immunotherapy protocols, with Yamauchi et al. and Qiu et al. separately reporting preliminary successes using atezolizumab combined with bevacizumab in treating hepatic angiosarcoma[ 33 , 34 ]. These individualized treatment strategies targeting specific molecular targets may provide new directions for comprehensive hepatic angiosarcoma treatment in the future. Despite continuous advancements in treatment methods, most hepatic angiosarcoma patients succumb to liver failure, intra-abdominal hemorrhage, and other complications within six months of diagnosis, with only 3% of patients surviving beyond two years[ 35 ]. In the present case, the patient demonstrated favorable short-term outcomes, and due to economic constraints and personal preferences, no further treatment was pursued after surgery. However, considering the high risk of recurrence, close follow-up and monitoring remain imperative. Based on current research, the therapeutic strategy for hepatic angiosarcoma should evolve towards a multidisciplinary comprehensive treatment approach. Future efforts must focus on developing more specific imaging diagnostic techniques and serological markers to enable early detection; establishing multicenter clinical studies to evaluate the long-term efficacy of different treatment strategies; and conducting in-depth investigations into molecular pathological mechanisms to identify new therapeutic targets. Combination immunotherapy with checkpoint inhibitors and antiangiogenic drugs, along with precision medicine targeting specific gene mutations, offer promising avenues for more effective management of this rare and highly aggressive disease. For cases complicated by KMS, a multidisciplinary team is recommended to develop individualized treatment protocols, with emergency surgical intervention when necessary. Conclusion Acute rupture of hepatic angiosarcoma with KMS is a rare and critically dangerous clinical condition requiring rapid and precise multidisciplinary intervention. In this case, emergency hepatectomy successfully not only effectively controlled life-threatening hemorrhage but also simultaneously resected the lesion causing KMS, securing a precious opportunity for patient survival. For clinical scenarios presenting with right upper quadrant pain accompanied by anemia, thrombocytopenia, and coagulation dysfunction, clinicians should maintain a high index of suspicion for hepatic angiosarcoma with KMS, promptly conducting imaging examinations and pathological assessments to develop a scientific diagnostic and treatment strategy at the earliest opportunity. Abbreviations PHA Primary Hepatic Angiosarcoma KMS Kasabach-Merritt Syndrome KMP Kasabach-Merritt Phenomenon TAE Transarterial Embolization TACE Transarterial Chemoembolization BRAF B-Raf Proto-Oncogene PD-1 Programmed Cell Death Protein 1 VEGF Vascular Endothelial Growth Factor Declarations Case report. This study was conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief . The authors declare that they have no conflict of interest. Ethics approval and consent to participate Ethical approval for this study was waived under the consideration of informed consent by the patient. Consent for publication This study was conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from the patient for publication of this case report and accompanying images. Competing interests The authors declare no competing interests. Funding This work was funded by Guilin Scientific Research and Technology Development Plan Project [2024] No. 17 [grant number 20230135-1-3]and the Guangxi Medical and Health Key Discipline Construction Project2021[ 8 ]. Author Contribution L.Y. and W.Y. contributed equally to this work as co-first authors. L.Y. conducted the clinical data collection and analysis, and wrote the main manuscript text. W.Y. performed the pathological examination and prepared the figures and tables. H.S. and H.Z. conducted the literature review and assisted with data interpretation. R.L. and J.W. were responsible for patient management and treatment. Y.Y. conceived and designed the study, supervised the project, and served as the corresponding author. All authors reviewed and approved the final manuscript. Acknowledgement The authors would like to express their heartfelt gratitude to Dr. Wang Shanhuan for providing valuable pathological materials and professional guidance for this case study. Data availability No datasets were generated or analysed during the current study References Rojas S, Rey Chaves CE, Robledo S, Conde D, Sabogal Olarte JC. Primary hepatic angiosarcoma: a systematic review. Ann Med Surg (Lond). 2024;86(3):1601–5. 10.1097/MS9.0000000000001584 . Durmaz S, Basak M, Ozguven BY, Eken KG, Erturk SM. Primary Hepatic Angiosarcoma: A Rare and Very Aggressive Liver Tumour. J Coll Physicians Surg Pak. 2022;32(12):SS209–11. 10.29271/jcpsp.2022.Supp.S209 . 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Cite Share Download PDF Status: Published Journal Publication published 22 Oct, 2025 Read the published version in World Journal of Surgical Oncology → Version 1 posted Editorial decision: Revision requested 12 Aug, 2025 Reviews received at journal 11 Aug, 2025 Reviews received at journal 03 Aug, 2025 Reviewers agreed at journal 01 Aug, 2025 Reviewers agreed at journal 01 Aug, 2025 Reviewers agreed at journal 20 Jul, 2025 Reviewers agreed at journal 18 Jul, 2025 Reviews received at journal 03 Jul, 2025 Reviewers agreed at journal 03 Jul, 2025 Reviewers invited by journal 08 May, 2025 Editor assigned by journal 01 May, 2025 Submission checks completed at journal 17 Apr, 2025 First submitted to journal 16 Apr, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-6461668","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":455594617,"identity":"36bdbfe9-273f-4ad9-afba-371c08941a93","order_by":0,"name":"Lin Ye","email":"","orcid":"","institution":"The Affiliated Hospital of Guilin Medical University","correspondingAuthor":false,"prefix":"","firstName":"Lin","middleName":"","lastName":"Ye","suffix":""},{"id":455594619,"identity":"04fab816-fe7b-4e8d-9f4a-69b1b614be7b","order_by":1,"name":"Wanrong Yue","email":"","orcid":"","institution":"Guilin People's Hospital","correspondingAuthor":false,"prefix":"","firstName":"Wanrong","middleName":"","lastName":"Yue","suffix":""},{"id":455594620,"identity":"45eebcad-ce81-4d70-abc6-2891188604a5","order_by":2,"name":"Hao Shi","email":"","orcid":"","institution":"The Affiliated Hospital of Guilin Medical University","correspondingAuthor":false,"prefix":"","firstName":"Hao","middleName":"","lastName":"Shi","suffix":""},{"id":455594622,"identity":"a706064a-77e7-468f-9f2c-fc8d8f15f6c1","order_by":3,"name":"Renjian Li","email":"","orcid":"","institution":"The Affiliated Hospital of Guilin Medical University","correspondingAuthor":false,"prefix":"","firstName":"Renjian","middleName":"","lastName":"Li","suffix":""},{"id":455594623,"identity":"5505c4af-fda1-4d21-bd55-29d960f14744","order_by":4,"name":"Jun Weng","email":"","orcid":"","institution":"The Affiliated Hospital of Guilin Medical University","correspondingAuthor":false,"prefix":"","firstName":"Jun","middleName":"","lastName":"Weng","suffix":""},{"id":455594626,"identity":"a4fb4fc6-683b-4f50-ba96-2a7c76ab7014","order_by":5,"name":"Haozhe Zhou","email":"","orcid":"","institution":"Guilin Medical University","correspondingAuthor":false,"prefix":"","firstName":"Haozhe","middleName":"","lastName":"Zhou","suffix":""},{"id":455594631,"identity":"987a7159-ff1d-446c-99ce-9fbcac57bac1","order_by":6,"name":"Yaqun Yu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA3UlEQVRIiWNgGAWjYJCCAwkVNnIM7GA2M1E6GB88OJNmDFVMnBZmw4dthxMbiNZicCN5m0QC2+H0+c7ciR8YKqwTG9jPHiCgJa1MIoEnPXfjYd7NEgxn0hMbePISCGjJMZNIkLDO3djMu42BEeRCCR4DIrQYMKcbgrX8I06LsUFCgnOCPDNISwMRWiTPPCt8kHAgzXADM9AvCcfSjdt4cvBr4TuevOHgz3828vLtvRs/fKixlu1nP4Nfi8KFBIgCgwNAIgGI2fCqBwL5/gMQLfINhJSOglEwCkbBiAUAK0BIsSB+v5MAAAAASUVORK5CYII=","orcid":"","institution":"The Affiliated Hospital of Guilin Medical University","correspondingAuthor":true,"prefix":"","firstName":"Yaqun","middleName":"","lastName":"Yu","suffix":""}],"badges":[],"createdAt":"2025-04-16 09:08:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6461668/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6461668/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12957-025-04046-z","type":"published","date":"2025-10-22T16:16:30+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":82621057,"identity":"aa182d33-4e2a-4a74-b323-1167fecdc48b","added_by":"auto","created_at":"2025-05-13 12:18:50","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":102617,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ePatient diagnosis and treatment flowchart.\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-6461668/v1/c3abba5f90d92b38d82a224a.png"},{"id":82621060,"identity":"33e7ae5c-8d7c-45c4-be24-e983478c9030","added_by":"auto","created_at":"2025-05-13 12:18:50","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1220508,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eClinical and radiological findings. \u003c/strong\u003eA. Multiple scattered purpuric lesions observed on the patient's abdomen and lower extremities, indicating coagulation abnormalities. B. Bedside FAST (Focused Assessment with Sonography for Trauma) ultrasound showing significant free fluid in Morrison's pouch (hepatorenal recess), consistent with hemoperitoneum. C-F. Contrast-enhanced abdominal computed tomography images. C and D demonstrate a large heterogeneous low-density mass (approximately 10.0×16.0 cm) in the posterior segment of the right liver lobe. E and F show heterogeneous enhancement of the mass during arterial phase with progressive filling in portal venous and delayed phases. Peritumoral fluid collection with a density of 45-50 HU and massive intraperitoneal fluid is evident, consistent with tumor rupture and active bleeding.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-6461668/v1/8c01ff7184edb81294964c3a.png"},{"id":82621064,"identity":"a82951fb-8126-4e83-b5a0-da6c34ce328a","added_by":"auto","created_at":"2025-05-13 12:18:51","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":2469530,"visible":true,"origin":"","legend":"\u003cp\u003ePathological examination of hepatic angiosarcoma. \u003cstrong\u003eA-B\u003c/strong\u003e. Gross specimen of the resected liver lobe showing a dark red, irregular mass with areas of hemorrhage and necrosis. The circular marking indicates the site of tumor rupture, with an approximate size of 8.5 cm×7.5 cm. \u003cstrong\u003eC\u003c/strong\u003e. Hematoxylin and Eosin (H\u0026amp;E) staining(×100) reveals atypical spindle-shaped tumor cells arranged in irregular sheets and bundles. \u003cstrong\u003eD-E\u003c/strong\u003e. Immunohistochemical staining for endothelial markers(×100): \u003cstrong\u003eD. \u003c/strong\u003eCD31: Demonstrates strong positive staining, confirming the vascular origin of the tumor. \u003cstrong\u003eE.\u003c/strong\u003e CD34: Also shows positive staining, further supporting the endothelial differentiation. \u003cstrong\u003eF\u003c/strong\u003e. Cytokeratin (CK) staining is negative, helping to rule out epithelial origin of the tumor. \u003cstrong\u003eG\u003c/strong\u003e. Ki-67 proliferation index: Approximately 25% of tumor cells show positive staining, indicating a high proliferation rate characteristic of malignant tumors.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-6461668/v1/1c0d8c27b5dcaba5f81db2e0.png"},{"id":94490602,"identity":"c763f25f-57e1-4348-b744-9b7521ddea72","added_by":"auto","created_at":"2025-10-27 17:12:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":4141513,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6461668/v1/48982210-52b0-4170-8495-0d24096111d9.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Acute Rupture of Primary Hepatic Angiosarcoma with Kasabach-Merritt Syndrome: A Rare Case Report and Literature Review","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePrimary hepatic angiosarcoma(PHA) is an extremely rare malignant tumor originating from endothelial cells, blood vessels, and lymphatic vessels, accounting for only 0.1-2% of all primary hepatic malignancies and 4% of all angiosarcomas. This tumor exhibits highly aggressive characteristics and is typically discovered at an advanced stage with multifocal lesions, resulting in a poor prognosis with a median survival of approximately 6 months [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Although the etiology remains incompletely understood, studies have indicated that exposure to vinyl chloride, thorium dioxide, arsenic, and long-term use of androgens may be associated with its development [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eKasabach-Merritt syndrome (KMS) was initially described in infant hemangiomas and is characterized by consumptive coagulopathy, manifesting as thrombocytopenia, hypofibrinogenemia, and microangiopathic hemolytic anemia[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. While KMS is commonly seen in infantile kaposiform hemangioendothelioma or tufted hemangioma, its occurrence in adult hepatic angiosarcoma is extremely rare[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eSpontaneous rupture of hepatic angiosarcoma is a life-threatening complication with an incidence of approximately 10\u0026ndash;25%[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. The mechanism of rupture may be related to the abnormal vascular structure of the tumor, its invasive growth pattern, and infiltration of adjacent blood vessels. When spontaneous rupture is accompanied by KMS, the clinical situation becomes even more critical, requiring prompt diagnosis and management[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn the published literature to date, cases of adult hepatic angiosarcoma with KMS and acute rupture reported are extremely rare. This paper reports a case of a 74-year-old female with acute rupture of hepatic angiosarcoma accompanied by KMS who was successfully treated with emergency right hemihepatectomy.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 74-year-old female with a 5-year history of well-controlled hypertension presented to our emergency department with \"sudden onset of severe right upper abdominal pain accompanied by nausea and vomiting for 3 hours.\" Two months prior, she had sought medical attention for fatigue and was found to have mild anemia (hemoglobin 103 g/L) and thrombocytopenia (platelet count 71\u0026times;10^9/L). At that time, she was diagnosed with \"anemia\" and prescribed oral iron supplements without further follow-up. Clinical details of the patient's diagnostic and treatment process are illustrated in Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eUpon admission, the patient appeared pale and in poor general condition. Vital signs showed: blood pressure 86/45 mmHg, heart rate 125 beats/min, respiratory rate 22 breaths/min, and oxygen saturation 92% (with supplemental oxygen). Physical examination revealed scattered purpura on the abdomen and lower extremities (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eA). Abdominal examination showed significant right upper quadrant tenderness with rebound pain and muscle guarding, enlarged hepatic dullness, and positive shifting dullness. Laboratory tests indicated: white blood cell count 15.2\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L, hemoglobin 76 g/L, platelet count 45\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L; prothrombin time 18.5 seconds, activated partial thromboplastin time 45.7 seconds, fibrinogen 1.2 g/L, D-dimer 3.5 mg/L; alanine aminotransferase 87 U/L, aspartate aminotransferase 156 U/L, total bilirubin 28 \u0026micro;mol/L, direct bilirubin 13 \u0026micro;mol/L, and albumin 29 g/L. Bedside ultrasound examination revealed large amounts of free fluid in Morrison's pouch (hepatorenal recess) and throughout the peritoneal cavity, consistent with hemoperitoneum(Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eB). Emergency contrast-enhanced abdominal CT revealed a low-density mass measuring approximately 10.0 cm \u0026times; 16.0 cm in the posterior segment of the right liver lobe, with heterogeneous enhancement in the arterial phase and progressive filling in the portal venous and delayed phases. Fluid collection with a density of approximately 45\u0026ndash;50 HU was observed around the mass, suggestive of blood. Massive intraperitoneal fluid was also noted (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eC-\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eF).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eGiven the clinical presentation of suspected hepatic tumor rupture leading to hypovolemic shock with coagulation dysfunction, immediate multidisciplinary assessment was performed. Initial resuscitation included intravenous fluids, blood product transfusions, and vasopressor support, followed by emergency exploratory laparotomy. Intraoperatively, approximately 3000 mL of bloody fluid and clots were found in the peritoneal cavity. A dark red mass measuring approximately 10 cm \u0026times; 9 cm was identified in the posterior segment of the right liver lobe, with visible rupture and active bleeding points on its surface. Based on these findings, a right hemihepatectomy was performed. Intraoperative blood loss was approximately 2500 mL, necessitating transfusion of 8 units of packed red blood cells and 800 mL of fresh frozen plasma.\u003c/p\u003e \u003cp\u003ePathological examination of the gross specimen (right liver lobe tissue measuring 16 cm \u0026times; 11 cm \u0026times; 8 cm) revealed a dark red mass measuring 8.5 cm \u0026times; 7.5 cm with indistinct borders. Portions of the mass had a sponge-like appearance with areas of necrosis and hemorrhage (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eA\u0026amp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eB). Microscopically, the tumor consisted of atypical spindle-shaped cells arranged in bundles or irregular sheets, forming irregular vascular spaces lined by atypical endothelial cells. The tumor cells exhibited large, hyperchromatic nuclei, scant cytoplasm, and frequent mitotic figures (7\u0026ndash;8 per 10 high-power fields) (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eC). Immunohistochemical studies showed: CD31(+), CD34(+), ERG(+), FLI-1(+), Ki-67 (approximately 25%+), CK(-), Hepatocyte(-), and GPC-3(-).(Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eD-\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eG). The pathological diagnosis was hepatic angiosarcoma.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003ePrimary Hepatic angiosarcoma(PHA) is an extremely rare malignant tumor originating from malignant proliferation of hepatic endothelial cells, characterized by high invasiveness and widespread distribution. Despite its low incidence, PHA is the most common primary mesenchymal malignancy of the liver in adults, accounting for less than 2% of all primary hepatic malignancies[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Epidemiological studies show that this disease predominantly affects males over 60 years of age, with a male-to-female ratio of 2\u0026ndash;4:1. Regarding the pathogenesis of PHA, current research has found that approximately 25% of cases are associated with environmental and occupational exposure factors, including vinyl chloride monomer, arsenic compounds, thorium dioxide (Thorotrast) contrast agents, synthetic steroids, as well as systemic diseases such as hemochromatosis and neurofibromatosis; while the etiology of the remaining 75% of cases remains unclear, possibly involving complex molecular pathological mechanisms. long-term exposure to vinyl chloride monomer and arsenic compounds is considered a significant risk factor for the development of hepatic angiosarcoma, which has been confirmed in epidemiological investigations of populations around industrial areas[\u003cspan additionalcitationids=\"CR11\" citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn this case, through detailed history taking, we discovered that the patient had worked in a plastic products factory for over 30 years, primarily responsible for processing and handling polyvinyl chloride products, with long-term exposure to a vinyl chloride monomer environment. This known causative factor for hepatic angiosarcoma likely played a significant role in the development of the disease in this patient. The interval from the patient's initial exposure to these carcinogens until the appearance of the tumor spanned several decades, consistent with the 10\u0026ndash;40 year latency period following vinyl chloride exposure reported in previous literature. This case once again emphasizes the importance of occupational and environmental exposure history in the diagnosis of unexplained vascular tumors of the liver, while suggesting that we should strengthen health monitoring and screening for populations with similar exposure histories.\u003c/p\u003e \u003cp\u003eThe clinical presentation of hepatic angiosarcoma is diverse and lacks specificity, which constitutes the primary reason for diagnostic difficulty and delay. According to the literature, abdominal pain is the most common chief complaint, with approximately 60% of patients initially presenting with abdominal pain as their primary symptom[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. This is followed by fatigue, weight loss, abdominal distension, anorexia, and fever\u0026mdash;nonspecific symptoms similar to those of other chronic liver diseases. Physical examination may reveal hepatomegaly, ascites, or jaundice, with approximately one-third of patients demonstrating obvious hepatic masses on initial diagnosis. Laboratory tests typically show abnormal liver function, but without specific changes; hepatitis B immunological testing is usually negative, and currently there are no reliable serological markers for early diagnosis. As the disease progresses, patients may develop serious complications such as cirrhosis and liver failure; due to the vascular nature of the tumor, approximately 17%-27% of patients experience spontaneous tumor rupture with hemoperitoneum, a life-threatening emergency typically presenting as sudden abdominal pain and hemorrhagic shock[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Additionally, some patients may develop KMS, characterized by thrombocytopenia, decreased fibrinogen, and consumptive coagulopathy. Hepatic angiosarcoma is highly malignant, with approximately 25%-40% of patients already exhibiting hematogenous metastases to organs such as the lungs, spleen, or bones at the time of diagnosis[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]. In this case, the patient initially presented with acute abdominal pain and shock, accompanied by laboratory findings indicating coagulation dysfunction and thrombocytopenia, and imaging revealing hepatic tumor rupture\u0026mdash;a combination of clinical presentations suggesting the possibility of hepatic angiosarcoma.\u003c/p\u003e \u003cp\u003eKMS, also known as Kasabach-Merritt phenomenon (KMP), is a rare complication of vascular tumors, initially described primarily in children with kaposiform hemangioendothelioma or tufted angioma, but has recently been reported in adult cases of hepatic angiosarcoma as well(Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e)[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. Research indicates that tumor size is one of the independent risk factors for the development of KMS, with truncal lesions more likely to develop KMS than non-truncal locations[\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. Ji et al. confirmed that approximately 70% of patients with kaposiform hemangioendothelioma presented with KMS, and that age at tumor discovery, morphology, and tumor size were independent risk factors for KMS development[\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSummary of Reported Cases of Hepatic Angiosarcoma with Kasabach-Merritt Syndrome.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"12\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c8\" colnum=\"8\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c9\" colnum=\"9\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c10\" colnum=\"10\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c11\" colnum=\"11\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c12\" colnum=\"12\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCase\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eAge\u003c/p\u003e \u003cp\u003e(years)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eSex\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSymptoms\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eViral Hepatitis\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eChemical Exposure\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003ePlatelet\u003c/p\u003e \u003cp\u003e(10\u003csup\u003e9\u003c/sup\u003e/L)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c8\"\u003e \u003cp\u003eFDP\u003c/p\u003e \u003cp\u003e(\u0026micro;g/mL)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c9\"\u003e \u003cp\u003eFibrinogen\u003c/p\u003e \u003cp\u003e(mg/dL)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c10\"\u003e \u003cp\u003eTreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c11\"\u003e \u003cp\u003eSurvival Time\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c12\"\u003e \u003cp\u003eCause of Death\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eWadhwa et al.\u003csup\u003e[5]\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e44\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eabdominal pain and jaundice\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e57\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e60\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eliver needle biopsy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFujii et al.\u003csup\u003e\u003cb\u003e[8]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e76\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eGingival bleeding\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e233\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e124\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eSupportive care\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e4 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eAbdominal bleeding\u003c/p\u003e \u003cp\u003eliver failure\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDeng et al.\u003csup\u003e\u003cb\u003e[16]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e59\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eHemoptysis\u003c/p\u003e \u003cp\u003enodules in lungs\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e90\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e56.22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e87\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003ePartial hepatectomy\u003c/p\u003e \u003cp\u003ePaclitaxel adjuvant chemotherapy (3 cycles)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e9 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eTumor metastasis\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eZhang et al.\u003csup\u003e\u003cb\u003e[19]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDisturbance of consciousness, jaundice\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e21\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e74.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e81.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eLiver transplantation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e2 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eTumor metastasis\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTang et al.\u003csup\u003e\u003cb\u003e[20]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e66\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRight upper abdominal pain\u003c/p\u003e \u003cp\u003emultiple skin purpuras\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e37\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e110.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e150\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eTACE\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e6 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eMultiple liver metastases\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGonz\u0026aacute;lez et al.\u003csup\u003e\u003cb\u003e[22]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e70\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRight hypochondrium pain and mass sensation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e42\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eRight hepatectomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCui et al.\u003csup\u003e\u003cb\u003e[23]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e70\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eChest tightness、Dyspnea\u003c/p\u003e \u003cp\u003eabdominal discomfort\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ePositive\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e95\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e127.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e154\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eSupportive care, attempted interventional radiology\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e2 weeks\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003ePulmonary hemorrhage\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTezuka et al.\u003csup\u003e\u003cb\u003e[25]\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNewborn\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eTachypnea、Cyanosis\u003c/p\u003e \u003cp\u003eHypotension\u003c/p\u003e \u003cp\u003eabdominal vascular murmur\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eNormal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e22.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e71\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eCorticosteroids, coil embolization\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e\u0026gt; 9 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eN/A\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eOur case\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e74\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eright upper abdominal pain\u003c/p\u003e \u003cp\u003emultiple cutaneous purpuras\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNegative\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eVinyl chloride exposure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e71\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e250\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e120\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eRight hepatectomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003e\u0026gt; 9 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eN/A\u003c/p\u003e \u003cp\u003e(To date, No recurrance )\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"12\" nameend=\"c12\" namest=\"c1\"\u003e \u003cp\u003eNS\u0026thinsp;=\u0026thinsp;Not Specified; N/A\u0026thinsp;=\u0026thinsp;Not Applicable; FDP\u0026thinsp;=\u0026thinsp;Fibrin Degradation Products; TACE\u0026thinsp;=\u0026thinsp;Transarterial Chemoembolization.\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThe pathophysiology of KMS remains incompletely elucidated, but is generally believed to be associated with tumor endothelial cells inducing platelet activation and aggregation, while local turbulence and abnormal vascular structures within the tumor may promote the coagulation cascade. Platelet sequestration and consumption within the tumor leads to thrombocytopenia; red blood cells are destroyed in irregular vascular spaces, resulting in microangiopathic hemolytic anemia; simultaneously, continuous coagulation activation depletes coagulation factors, causing hypofibrinogenemia and coagulation dysfunction. Notably, while liver failure is commonly caused by drug toxicity and viral hepatitis, liver failure induced by hepatic angiosarcoma is relatively rare[\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn the present case, the patient had experienced fatigue accompanied by anemia and thrombocytopenia two months prior to admission, suggesting that the tumor and KMS may have been present for some time. Based on the patient's coagulation abnormalities and thrombocytopenia upon admission, combined with clinical symptoms, we diagnosed KMS. Regarding the radiological diagnosis of hepatic angiosarcoma, typical features include heterogeneous enhancement in the arterial phase with progressive filling in the portal venous and delayed phases, but these features have limited specificity. The hepatic mass shown on the emergency CT scan of this patient corresponded with these characteristics, but due to the emergency situation, more detailed examinations could not be completed, and a definitive diagnosis of hepatic angiosarcoma could not be established preoperatively. The final diagnosis of hepatic angiosarcoma with KMS was confirmed through surgery and pathological examination. The gradual normalization of the patient's coagulation function and platelet count postoperatively further confirmed the causal relationship between these two conditions.\u003c/p\u003e \u003cp\u003eTherapeutic options for hepatic angiosarcoma are limited, with surgical resection currently remaining the only potentially curative method. However, due to the disease's invasive nature, most patients are diagnosed at an advanced stage, with research indicating that only approximately 20% of patients are suitable for surgical intervention. The invasive characteristics and multifocality of angiosarcoma further restrict surgical applications, with partial hepatectomy typically considered only when the lesion is confined to a single hepatic lobe. Nevertheless, surgical resection significantly improves patient prognosis[\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e, \u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eWilson et al.'s research found that patients undergoing surgical resection had a median survival of 33.4 months, significantly superior to non-surgical patients[\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. Mart\u0026iacute;nez et al.'s retrospective study similarly demonstrated that surgically treated patients had a median survival of 8 months, compared to merely 2 months for those without surgical intervention[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]. For the patient in this case, emergency surgical treatment is both reasonable and necessary due to tumor rupture accompanied by hemodynamic instability.\u003c/p\u003e \u003cp\u003eIn critical situations of tumor rupture with intra-abdominal hemorrhage, transarterial embolization (TAE) is the preferred method for controlling life-threatening bleeding, though it carries risks of rebleeding and poor prognosis[\u003cspan additionalcitationids=\"CR26\" citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]. Liver transplantation was previously considered a treatment option, but due to high post-operative recurrence rates and distant metastasis risks, it is currently rarely recommended. However, individual successful cases merit attention, such as the report by Dhaliwal et al. of a 61-year-old female patient who showed no tumor recurrence 16 months after liver transplantation, suggesting that transplantation might offer a treatment opportunity for select patients under specific circumstances[\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eFor unresectable cases, treatment options are even more limited. Studies indicate that hepatic angiosarcoma demonstrates poor responsiveness to radiation therapy, exhibiting significant radioresistance[\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e]. Regarding chemotherapy, drugs such as docetaxel, paclitaxel, and doxorubicin have shown certain efficacy, particularly in advanced-stage patients[\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e]. Systematic studies reveal that patients receiving chemotherapy have longer survival times and lower mortality risks compared to those receiving no chemotherapy, though the overall effect remains unsatisfactory, indicating that current treatment strategies still require improvement[\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e, \u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn recent years, molecular targeted therapy and immunotherapy have brought new hope for hepatic angiosarcoma patients. Gera et al. discovered BRAF mutations in some hepatic angiosarcoma patients, with those patients demonstrating excellent responses to the BRAF inhibitor vemurafenib. Additionally, pazopanib, PD-1 inhibitors, and therapies targeting vascular endothelial growth factor (VEGF) have shown research progress[\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e]. Lin et al. reported successful cases of transarterial chemoembolization (TACE) combined with targeted immunotherapy[\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]. Even more encouragingly, recent studies have explored combination immunotherapy protocols, with Yamauchi et al. and Qiu et al. separately reporting preliminary successes using atezolizumab combined with bevacizumab in treating hepatic angiosarcoma[\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e, \u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]. These individualized treatment strategies targeting specific molecular targets may provide new directions for comprehensive hepatic angiosarcoma treatment in the future.\u003c/p\u003e \u003cp\u003eDespite continuous advancements in treatment methods, most hepatic angiosarcoma patients succumb to liver failure, intra-abdominal hemorrhage, and other complications within six months of diagnosis, with only 3% of patients surviving beyond two years[\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e]. In the present case, the patient demonstrated favorable short-term outcomes, and due to economic constraints and personal preferences, no further treatment was pursued after surgery. However, considering the high risk of recurrence, close follow-up and monitoring remain imperative.\u003c/p\u003e \u003cp\u003eBased on current research, the therapeutic strategy for hepatic angiosarcoma should evolve towards a multidisciplinary comprehensive treatment approach. Future efforts must focus on developing more specific imaging diagnostic techniques and serological markers to enable early detection; establishing multicenter clinical studies to evaluate the long-term efficacy of different treatment strategies; and conducting in-depth investigations into molecular pathological mechanisms to identify new therapeutic targets. Combination immunotherapy with checkpoint inhibitors and antiangiogenic drugs, along with precision medicine targeting specific gene mutations, offer promising avenues for more effective management of this rare and highly aggressive disease. For cases complicated by KMS, a multidisciplinary team is recommended to develop individualized treatment protocols, with emergency surgical intervention when necessary.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eAcute rupture of hepatic angiosarcoma with KMS is a rare and critically dangerous clinical condition requiring rapid and precise multidisciplinary intervention. In this case, emergency hepatectomy successfully not only effectively controlled life-threatening hemorrhage but also simultaneously resected the lesion causing KMS, securing a precious opportunity for patient survival. For clinical scenarios presenting with right upper quadrant pain accompanied by anemia, thrombocytopenia, and coagulation dysfunction, clinicians should maintain a high index of suspicion for hepatic angiosarcoma with KMS, promptly conducting imaging examinations and pathological assessments to develop a scientific diagnostic and treatment strategy at the earliest opportunity.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003ePHA\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003ePrimary Hepatic Angiosarcoma\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eKMS\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eKasabach-Merritt Syndrome\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eKMP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eKasabach-Merritt Phenomenon\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eTAE\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eTransarterial Embolization\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eTACE\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eTransarterial Chemoembolization\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eBRAF\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eB-Raf Proto-Oncogene\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003ePD-1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eProgrammed Cell Death Protein 1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eVEGF\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\"\u003e\n \u003cp\u003eVascular Endothelial Growth Factor\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e"},{"header":"Declarations","content":"\u003cp\u003eCase report.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThis study was conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief .\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no conflict of interest.\u0026nbsp;\u003c/p\u003e \u003cp\u003e \u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e \u003cp\u003eEthical approval for this study was waived under the consideration of informed consent by the patient.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eThis study was conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from the patient for publication of this case report and accompanying images.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eCompeting interests\u003c/strong\u003e \u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eThis work was funded by Guilin Scientific Research and Technology Development Plan Project [2024] No. 17 [grant number 20230135-1-3]and the Guangxi Medical and Health Key Discipline Construction Project2021[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eL.Y. and W.Y. contributed equally to this work as co-first authors. L.Y. conducted the clinical data collection and analysis, and wrote the main manuscript text. W.Y. performed the pathological examination and prepared the figures and tables. H.S. and H.Z. conducted the literature review and assisted with data interpretation. R.L. and J.W. were responsible for patient management and treatment. Y.Y. conceived and designed the study, supervised the project, and served as the corresponding author. All authors reviewed and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eThe authors would like to express their heartfelt gratitude to Dr. Wang Shanhuan for providing valuable pathological materials and professional guidance for this case study.\u003c/p\u003e\u003ch2\u003eData availability\u003c/h2\u003e \u003cp\u003eNo datasets were generated or analysed during the current study\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eRojas S, Rey Chaves CE, Robledo S, Conde D, Sabogal Olarte JC. Primary hepatic angiosarcoma: a systematic review. 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BMC Surg. 2019;19(1):5. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1186/s12893-018-0465-5\u003c/span\u003e\u003cspan address=\"10.1186/s12893-018-0465-5\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"world-journal-of-surgical-oncology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"wjso","sideBox":"Learn more about [World Journal of Surgical Oncology](http://wjso.biomedcentral.com)","snPcode":"12957","submissionUrl":"https://submission.nature.com/new-submission/12957/3","title":"World Journal of Surgical Oncology","twitterHandle":"@OncoBioMed","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Hepatic angiosarcoma, Kasabach-Merritt syndrome, Acute rupture, Emergency hepatectomy, Coagulopathy","lastPublishedDoi":"10.21203/rs.3.rs-6461668/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6461668/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e Primary hepatic angiosarcoma (PHA) is an extremely rare malignant tumor originating from vascular endothelial cells, accounting for only 0.1-2% of primary liver malignancies. Kasabach-Merritt syndrome (KMS) is a rare complication characterized by thrombocytopenia, coagulation dysfunction, and microangiopathic hemolytic anemia, seldom reported in adult patients with hepatic angiosarcoma.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation\u003c/strong\u003e We describe a 74-year-old female with a history of vinyl chloride exposure who presented with acute right upper quadrant pain, shock, and scattered purpura. Laboratory tests revealed thrombocytopenia and coagulation abnormalities. Imaging demonstrated a large ruptured hepatic mass with hemoperitoneum. The patient underwent emergency right hemihepatectomy, with pathology confirming hepatic angiosarcoma. Postoperatively, the patient's coagulation parameters normalized, confirming resolution of KMS. The patient showed no recurrence at 9-month follow-up.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e Acute rupture of hepatic angiosarcoma with KMS is a life-threatening condition requiring rapid multidisciplinary intervention. Emergency hepatectomy not only controlled the hemorrhage but also removed the lesion causing KMS. Clinicians should maintain a high index of suspicion for hepatic angiosarcoma in patients presenting with spontaneous hepatic hemorrhage and coagulation abnormalities.\u003c/p\u003e","manuscriptTitle":"Acute Rupture of Primary Hepatic Angiosarcoma with Kasabach-Merritt Syndrome: A Rare Case Report and Literature Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-13 12:18:46","doi":"10.21203/rs.3.rs-6461668/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-08-12T04:11:44+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-11T07:03:12+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-04T02:08:43+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"91525855787331046537312089293083914429","date":"2025-08-01T04:42:50+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"266523154976614871606501774760275456986","date":"2025-08-01T04:38:01+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"232479108735284709245007194248299998253","date":"2025-07-21T03:17:27+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"215156822142605047313276210146362555545","date":"2025-07-18T18:03:53+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-07-03T20:18:22+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"237612735496299609313395381872612219054","date":"2025-07-03T19:56:40+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-05-08T09:18:56+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-05-02T03:00:49+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-04-17T12:15:47+00:00","index":"","fulltext":""},{"type":"submitted","content":"World Journal of Surgical Oncology","date":"2025-04-16T08:54:55+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"world-journal-of-surgical-oncology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"wjso","sideBox":"Learn more about [World Journal of Surgical Oncology](http://wjso.biomedcentral.com)","snPcode":"12957","submissionUrl":"https://submission.nature.com/new-submission/12957/3","title":"World Journal of Surgical Oncology","twitterHandle":"@OncoBioMed","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"3dd86ae2-2156-467a-bf6c-5f77b7a3ccf2","owner":[],"postedDate":"May 13th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-27T16:32:16+00:00","versionOfRecord":{"articleIdentity":"rs-6461668","link":"https://doi.org/10.1186/s12957-025-04046-z","journal":{"identity":"world-journal-of-surgical-oncology","isVorOnly":false,"title":"World Journal of Surgical Oncology"},"publishedOn":"2025-10-22 16:16:30","publishedOnDateReadable":"October 22nd, 2025"},"versionCreatedAt":"2025-05-13 12:18:46","video":"","vorDoi":"10.1186/s12957-025-04046-z","vorDoiUrl":"https://doi.org/10.1186/s12957-025-04046-z","workflowStages":[]},"version":"v1","identity":"rs-6461668","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6461668","identity":"rs-6461668","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}