Primary splenic hemangiosarcoma with liver metastasis and liver rupture after surgery: a rare case and literature review

Primary splenic hemangiosarcoma with liver metastasis and liver rupture after surgery: a rare case 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 Primary splenic hemangiosarcoma with liver metastasis and liver rupture after surgery: a rare case and literature review Zhidan Wu, Xiangming Cao, Yuanyuan Zuo, Yongqiang Zheng This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6348464/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Primary splenic angiosarcoma is an uncommon form of malignant splenic neoplasm that is characterized by rapid proliferation, significant invasiveness, and widespread metastases. However, the disease often presents with non-specific clinical manifestations, which can result in delayed diagnoses and misdiagnoses. Early diagnosis and intervention are therefore crucial for improving patient survival. Case Presentation This case report provides a comprehensive account of the diagnosis and treatment of a patient with primary splenic angiosarcoma, encompassing the entire clinical trajectory of the disease, including its onset, surgical intervention, postoperative chemotherapy, and ultimately, the patient's demise due to liver metastasis and liver rupture. The report is comprehensive and meticulously detailed, thus making it a valuable reference source. Prior to the onset of the disease, the patient exhibited no specific symptoms except for a decrease in platelets. CT and MRI played an important role in the diagnosis and evaluation of the disease. Surgical removal of the spleen, in conjunction with the formulation of an empirical chemotherapy regimen, enabled the patient to survive for a period of six months following surgery. However, the patient ultimately succumbed to a liver rupture. Conclusion Primary splenic angiosarcoma is a highly lethal condition, and prompt diagnosis and intervention are imperative for extending patients' survival. This case underscores the need for clinicians to exercise vigilance when encountering patients with prolonged thrombocytopenia, to utilize diverse diagnostic modalities to establish a differential diagnosis, and to endeavor to resect the tumour surgically while it is still small or, at the very least, before the spleen ruptures. Concurrently, there is an urgent need to develop new and more representative serum markers to facilitate differential diagnosis of the disease. Splenic tumour Angiosarcoma Splenic rupture Liver rupture Figures Figure 1 Figure 2 Figure 3 Figure 4 Introduction Primary splenic angiosarcoma (PSA) is an extremely rare primary malignant vascular tumour originating from the splenic vascular endothelium, especially the mesenchymal-derived vascular endothelial cells within the splenic sinus network. The incidence of PSA is approximately 0.14–0.23 per million, with the majority of cases occurring in the 50–79 age range, and exhibiting a male-to-female ratio of approximately 4:3 [ 1 , 2 ]. Since Langhans' initial report of PSA in 1879 [ 3 ], the English literatures have documented less than 300 cases. PSA accounts for almost 7.4% of all primary malignant splenic tumours, and it is an aggressive tumour with a high local recurrence and distant metastasis rate, which is easily misdiagnosed due to its low incidence and lack of obvious features in its early clinical manifestations, and it is usually found in advanced stages. The challenges in accurate diagnosis are compounded by the rarity of the condition and the absence of distinctive early clinical manifestations. This case report details a patient with PSA complicated by splenic rupture, who succumbed to liver metastasis and rupture following four and a half courses of conventional chemotherapy subsequent to surgical resection of the spleen. Case presentation The patient, a 52-year-old male, was admitted to the hospital on 8 August 2023, after experiencing left-sided abdominal discomfort and syncope without any obvious triggers seven hours earlier. The patient was admitted to the hospital with hypotensive shock and was treated symptomatically with blood transfusion and fluid replacement. Following stabilization of the patient's vital signs, an advanced CT examination was conducted, which revealed multiple splenic lesions, stroke, substantial abdominal and pelvic blood accumulation, multiple cysts in the liver, and a possible small hemangioma in the right lobe of the liver (Fig. 1 ). On physical examination, the abdomen was found to be soft and slightly distended, the liver and spleen were not palpated subcostally, and the abdomen sounded bulbous on percussion. Furthermore, a blood routine test revealed a haemoglobin level of 90 g/L and a platelet count of 23*10 9 /L. Concurrently, the serum levels of the tumour markers CEA, CA125, CA153, CA199, and CA242 were all found to be within the standard range. The patient had a past medical history of thrombocytopenia, which had not been given due consideration. The following night, the patient exhibited symptoms including pallor, profuse sweating, and a decrease in blood pressure. Given the splenic rupture and bleeding, the patient underwent a total splenectomy under general anaesthesia in an emergency. This procedure resulted in the accumulation of a large volume of blood in the abdominal cavity, estimated at approximately 2,000 ml. The spleen was palpably enlarged, with a hard surface and uneven texture, and a dark red-grayish cut surface, partly in the form of nodules, with indistinct boundaries, and two rupture openings were seen on the surface, The length of the object is approximately 6 centimetres. Postoperative pathology showed that the spleen was a tumour of vascular origin (Fig. 2 ), and the combination of immunohistochemistry was consistent with hemangiosarcoma (Fig. 3 ),CKAE1/AE3 (-), Vimentin (+), SMA (-), Desmin (-), S-100 (-), CD31 (+), CD34 (+), ALK p80 (-), ERG (+), INI-1(+), D2-40(-), CK7(-), and Ki-67(60%+) was identified. Postoperative complications were absent, the incision exhibited optimal recovery, and the patient was discharged from the hospital within 10 days. The final diagnosis was as follows: 1. splenic malignant tumour; 2. nontraumatic splenic rupture; 3. abdominal hemoperitoneum; and 4. grade 1 hypertension. On 29 September, the patient was admitted to the oncology department of our hospital, which specializes in both traditional Chinese and Western medicine. Following a thorough examination, no obvious contraindications to chemotherapy were identified. The following day, the patient was administered 150 mg of paclitaxel liposome intravenously on days 1 and 8. A total of three and a half courses were maintained. On January 14, 2024, a CT scan revealed the presence of multiple liver tumour metastases. In comparison with the MRI slice on August 21, 2022 (Fig. 4 ), the patient's liver disease progression was taken into consideration, and oral Sunitinib 50 mg qd targeted therapy was initiated on January 14th. On 10 February 2024, the patient presented with acute abdominal pain, primarily in the upper abdomen and right epigastrium. He was subsequently transferred to the emergency department, where a CT scan revealed a ruptured liver with concomitant bleeding. The patient and his family convened to discuss the circumstances and considered the option of no active treatment, opting instead for automatic discharge. The patient was subsequently discharged and died shortly thereafter. Discussion As is well established in the literature, PSA is a lethal tumour with an unknown pathogenic mechanism, and some environmental factors (such as chemical substances such as vinyl chloride monomer, thorium dioxide, arsenic, or radiation exposure) are considered risk factors for splenic vascular sarcoma [ 4 ]. One theory postulates that PSA may be the result of the malignant transformation of other benign spleen tumours, such as hemangiomas, angio endotheliomas and lymphangiomas [ 5 ]. The patient in this case denied a history of prior chemical exposure or benign spleen tumours. Early diagnosis of PSA is challenging due to its low incidence and tendency to be overlooked in broad differential diagnoses. Definitive diagnosis of PSA relies on puncture biopsy or pathology, but spleen biopsy is contraindicated due to the high risk of induced rupture and metastasis and the poor diagnostic yield [ 6 ]. The patient had been divorced for many years, had no regular occupation, had an irregular and poor health awareness, and never attended health check-ups. On 21 August 2022, the patient underwent an MRI scan of the upper abdomen due to abdominal pain, which revealed diffuse splenic space occupation. The size of the biggest nodule was approximately 5.0*6.0 cm, and the patient was considered to have a large vasculogenic lesion, multiple cysts of the liver and hemangiomas, which were not further diagnosed at that time. On 8 August 2023, the patient was admitted to hospital with acute abdominal pain, he did not undergo an ultrasound examination, instead, a CT scan of the abdomen and pelvis was performed. This revealed a splenic rupture, and the patient was immediately taken for emergency surgery to remove the spleen. Imaging played a crucial role in preoperative diagnosis, allowing valuable time for emergency surgery. Anemia is the primary manifestation of PSA laboratory tests, with others including leukopenia, thrombocytopenia, and increased erythrocyte sedimentation rate [ 7 ]. In this case, the patient exhibited atypical symptoms and presented to the local health center with acute abdomen. CT revealed pelvic effusion, leading to referral to a higher-level hospital. Laboratory tests for anemia and platelet reduction up to a critical value (23*10 9 /L) were conducted. During the diagnostic process and subsequent treatment, routine tumour marker examinations revealed elevated neuron-specific enolase levels (18.5 ng/ml; reference range < 16.3 ng/ml), with no significant anomalies observed in other indicators. The final diagnosis of this patient with PSA relied on pathological examination, and in addition to morphological features, previous studies [ 1 ] have shown that diagnosis of this tumour requires a combination of immunohistochemical findings, and immunohistochemical testing in this patient suggested that vascular differentiation was positive for CD34 and CD31, suggesting that Vimentin, which may have originated from mesenchymal tissue, was positive, consistent with the report. In cases of PSA, given its extremely low incidence, the treatment of choice is splenectomy, which has been shown to prolong survival, both in cases of invasive disseminated metastases and in those where splenectomy is performed before splenic rupture [ 8 ]. Lumpectomy is not the preferred option for most physicians, given that splenomegaly increases the risk of hemorrhage, pericardium rupture, and intermediate conversion to open laparotomy. In this case, due to the occurrence of acute symptoms such as a drop in blood pressure, the surgeon considered the possibility of splenic rupture, and subsequently performed open laparotomy with splenectomy, resulting in no postoperative complications. However, there are no specific chemotherapeutic regimens for splenic angiosarcoma, and a paucity of clinical evidence from large-scale studies, with most chemotherapeutic regimens being empirically designed. Vakkalanka et al.[ 9 ] discovered that the utilisation of paclitaxel for the treatment of patients with PSA can substantially enhance their prognoses. As found by de Azevedo OS et al.[ 10 ], patients with PSA demonstrated a positive response to taxane treatment, with the treatment effectively prolonging survival time. The patient was administered a paclitaxel liposome 150 mg d1,d8 intravenous chemotherapy regimen, which was continued for three and a half courses. A review of the literature reveals that the average survival time for patients with PSA after surgery is approximately six months. The fact that the patient in this case developed a liver rupture precisely six months after surgery, and had already exhibited liver metastases prior to his demise, serves to underscore the highly aggressive nature of the disease. Conclusion PSA is an uncommon disease with a high degree of malignancy and an absence of clinical features, necessitating vigilance on the part of clinicians when encountering patients with long-term splenomegaly, anemia, and thrombocytopenia. In such cases, a comprehensive differential diagnosis should be sought, with surgical resection of the tumour being the optimal approach when it is still small, or at least before splenic rupture, with the aim of maximizing patient survival. Future research could concentrate on elucidating the underlying molecular mechanisms that drive the development and progression of this disease. By elucidating these mechanisms, researchers may be able to identify specific serum markers to facilitate the differential diagnosis of PSA, and to develop more targeted therapies to improve patient outcomes. Abbreviations PSA Primary splenic angiosarcoma Declarations Acknowledgements The patient was a distant relative of mine and we thank the deceased and his family for their willingness to share this case for scientific and educational purposes. We thank the medical, imaging, and pathology teams involved in the diagnosis and treatment of this rare tumour for their dedication and expertise in ensuring the best possible outcome for the patient. Authors’ contributions Wu Z.D conceived and designed the study and drafted the manuscript. Cao X.M participated in the revision of the content related to clinical diagnosis and treatment. Zuo Y.Y provided clear routine pathology and immunohistochemistry image reports. Zheng Y.Q provided clear imaging image reports. All authors read and approved the manuscript. Funding This study was funded by Jiangyin Municipal Health Commission. Clinical Trial Registration This study is a retrospective case study report based on available medical data and does not involve active intervention in subjects. Consequently, clinical trial registration is not applicable. Data availability No datasets were generated or analysed during the current study. Ethical Statement This case report was approved by the Ethics Committee of Jiangyin People's Hospital in accordance with the Measures for Ethical Review of Life Science and Medical Research Involving Human Beings promulgated by the National Health and Health Commission. Written informed consent was obtained from the patient's family for the publication of this report (including all clinical details and pictures). All personally identifiable information has been removed to ensure patient confidentiality. Consent to participate The patient had already passed away by the time the author was considering putting the case together for publication. So written informed consent was obtained from the parents. They have consented to the submission of the case report for submission to the journal. Consent to publish Written informed consent for publication of clinical details and imaging was obtained from the patient's family. Competing interests The authors declare no competing interests. Data availability statements All data supporting the findings of this study are available within the paper and its Supplementary Information. References Li, R., et al., Clinical Characteristics and Prognostic Factors of Primary Splenic Angiosarcoma: A Retrospective Clinical Analysis from China. Cell Physiol Biochem, 2018. 49(5): p. 1959-1969. Cao, L., et al., A primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile. Medicine (Baltimore), 2019. 98(28): p. e16245. Falk, S., J. Krishnan, and J.M. Meis, Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. Am J Surg Pathol, 1993. 17(10): p. 959-70. Kamocki, Z., et al., Primary splenic angiosarcoma - the same diagnosis yielding two different clinical pictures. Case report. Contemp Oncol (Pozn), 2013. 17(2): p. 218-21. Batouli, A., et al., Primary Splenic Angiosarcoma: Clinical and Imaging Manifestations of This Rare Aggressive Neoplasm. Curr Probl Diagn Radiol, 2016. 45(4): p. 284-7. Fiorentino, M.D., et al., Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum. Autops Case Rep, 2019. 9(3): p. e2019100. Chen, X., et al., Early detection and integral resection are keys to extend survival in patients suffered from primary angiosarcoma of the spleen: A care-compliant case report and literature review. Medicine (Baltimore), 2018. 97(5): p. e9718. Duan, Y.F., et al., Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review. World J Surg Oncol, 2013. 11: p. 53. Vakkalanka, B. and M. Milhem, Paclitaxel as neoadjuvant therapy for high grade angiosarcoma of the spleen: a brief report and literature review. Clin Med Insights Oncol, 2010. 4: p. 107-10. de Azevedo, O.S., et al., Splenic Angiosarcoma: A Diagnostic Splenectomy Finding. Case Rep Oncol, 2016. 9(3): p. 733-737.This case report serves as a reminder of the importance of continued research into PSA. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted 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. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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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-6348464","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":453251209,"identity":"3dad9a9a-9df0-4fda-9239-4bb7c313d361","order_by":0,"name":"Zhidan Wu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA9klEQVRIiWNgGAWjYHACNoYENhDNw3CAgcEGyGBsPECKljSQlgbCWhigWoDgMFgIrxaDG+nPHjwos8tjYD978HBBzXm7te2HgbbU2ETj1pKQbpBwLrmYgScv4fCMY7eTt51JBGo5lpbbgEOL2Y2EYxKJbcyJDRI8Bod52G4nmx0AamFsOIxHS2IbUEs9VMu/c8lm5x8S0pLMBtRyGKKFt+2AHdAQ/Frszzxjk0g4dzyxjScHqKUvOcHsBtCWBDx+kWxPfyb5o6w6sZ/9jPFnnm929mbn0x8++FBjg1MLHLBB6USwygRCylFcSoriUTAKRsEoGBkAAGK7Y1u4GA2iAAAAAElFTkSuQmCC","orcid":"","institution":"Jiangyin People’s Hospital","correspondingAuthor":true,"prefix":"","firstName":"Zhidan","middleName":"","lastName":"Wu","suffix":""},{"id":453251210,"identity":"17f29a64-d0f8-4652-b2c9-183be12f6e7f","order_by":1,"name":"Xiangming Cao","email":"","orcid":"","institution":"Jiangyin People’s Hospital","correspondingAuthor":false,"prefix":"","firstName":"Xiangming","middleName":"","lastName":"Cao","suffix":""},{"id":453251211,"identity":"b8e728c3-f7fc-4482-bb08-15eb5306a8b0","order_by":2,"name":"Yuanyuan Zuo","email":"","orcid":"","institution":"Jiangyin People’s Hospital","correspondingAuthor":false,"prefix":"","firstName":"Yuanyuan","middleName":"","lastName":"Zuo","suffix":""},{"id":453251213,"identity":"63732ffb-7756-489d-91b2-6214925abae2","order_by":3,"name":"Yongqiang Zheng","email":"","orcid":"","institution":"Jiangyin People’s Hospital","correspondingAuthor":false,"prefix":"","firstName":"Yongqiang","middleName":"","lastName":"Zheng","suffix":""}],"badges":[],"createdAt":"2025-04-01 01:53:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6348464/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6348464/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":82355405,"identity":"c1ccac32-3410-4cb6-a384-8ac27f1b199a","added_by":"auto","created_at":"2025-05-09 11:14:24","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":158782,"visible":true,"origin":"","legend":"\u003cp\u003eCT image of the spleen showing multiple nodular occupations\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-6348464/v1/598aac9ef8b657a992b4be4a.png"},{"id":82355420,"identity":"eb551574-e776-404b-aed0-85e76f2955e5","added_by":"auto","created_at":"2025-05-09 11:14:24","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":637492,"visible":true,"origin":"","legend":"\u003cp\u003eHematoxylin and eosin (H\u0026amp;E) staining reveals regular nodules consisted of hyperplastic, dilated blood vessels with lumens of varying sizes, some with hemorrhage and necrosis.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-6348464/v1/392a951f2e97f8a659b75efb.png"},{"id":82355408,"identity":"f2ad6418-d3f7-4257-ac24-f8eea3840a68","added_by":"auto","created_at":"2025-05-09 11:14:24","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":498375,"visible":true,"origin":"","legend":"\u003cp\u003eImmunohistochemical characterization reveals (A)Tumour cells diffusely expressed CD31(+) (original magnifcation×400). (B)Tumour cells diffusely expressed CD34(+) (original magnifcation×400). (C)Tumour cells diffusely expressed EGR (+) (original magnifcation×400). (D)Tumour cells diffusely expressed INI-1(+) (original magnifcation×400).\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-6348464/v1/92e41e61471de3b2befea223.png"},{"id":82357582,"identity":"21feedf0-13d0-4766-a998-fd4fd9e7b42d","added_by":"auto","created_at":"2025-05-09 11:22:24","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":145051,"visible":true,"origin":"","legend":"\u003cp\u003eMRI images showed diffuse splenic occupancy, multiple cysts in the liver, and hemangiomas in the left inner and right posterior lobes of the liver.\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-6348464/v1/1f054181f5ca7c36ccfdda89.png"},{"id":83977225,"identity":"1710cf0a-1005-4d9b-a823-5ed6de76b01f","added_by":"auto","created_at":"2025-06-05 09:17:10","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1873802,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6348464/v1/9ef72d11-a592-4be8-b6f6-e1a2458c219a.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Primary splenic hemangiosarcoma with liver metastasis and liver rupture after surgery: a rare case and literature review","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePrimary splenic angiosarcoma (PSA) is an extremely rare primary malignant vascular tumour originating from the splenic vascular endothelium, especially the mesenchymal-derived vascular endothelial cells within the splenic sinus network. The incidence of PSA is approximately 0.14\u0026ndash;0.23 per million, with the majority of cases occurring in the 50\u0026ndash;79 age range, and exhibiting a male-to-female ratio of approximately 4:3 [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Since Langhans' initial report of PSA in 1879 [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e], the English literatures have documented less than 300 cases. PSA accounts for almost 7.4% of all primary malignant splenic tumours, and it is an aggressive tumour with a high local recurrence and distant metastasis rate, which is easily misdiagnosed due to its low incidence and lack of obvious features in its early clinical manifestations, and it is usually found in advanced stages. The challenges in accurate diagnosis are compounded by the rarity of the condition and the absence of distinctive early clinical manifestations. This case report details a patient with PSA complicated by splenic rupture, who succumbed to liver metastasis and rupture following four and a half courses of conventional chemotherapy subsequent to surgical resection of the spleen.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eThe patient, a 52-year-old male, was admitted to the hospital on 8 August 2023, after experiencing left-sided abdominal discomfort and syncope without any obvious triggers seven hours earlier. The patient was admitted to the hospital with hypotensive shock and was treated symptomatically with blood transfusion and fluid replacement. Following stabilization of the patient's vital signs, an advanced CT examination was conducted, which revealed multiple splenic lesions, stroke, substantial abdominal and pelvic blood accumulation, multiple cysts in the liver, and a possible small hemangioma in the right lobe of the liver (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). On physical examination, the abdomen was found to be soft and slightly distended, the liver and spleen were not palpated subcostally, and the abdomen sounded bulbous on percussion. Furthermore, a blood routine test revealed a haemoglobin level of 90 g/L and a platelet count of 23*10\u003csup\u003e9\u003c/sup\u003e/L. Concurrently, the serum levels of the tumour markers CEA, CA125, CA153, CA199, and CA242 were all found to be within the standard range. The patient had a past medical history of thrombocytopenia, which had not been given due consideration.\u003c/p\u003e \u003cp\u003eThe following night, the patient exhibited symptoms including pallor, profuse sweating, and a decrease in blood pressure. Given the splenic rupture and bleeding, the patient underwent a total splenectomy under general anaesthesia in an emergency. This procedure resulted in the accumulation of a large volume of blood in the abdominal cavity, estimated at approximately 2,000 ml. The spleen was palpably enlarged, with a hard surface and uneven texture, and a dark red-grayish cut surface, partly in the form of nodules, with indistinct boundaries, and two rupture openings were seen on the surface, The length of the object is approximately 6 centimetres. Postoperative pathology showed that the spleen was a tumour of vascular origin (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), and the combination of immunohistochemistry was consistent with hemangiosarcoma (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e),CKAE1/AE3 (-), Vimentin (+), SMA (-), Desmin (-), S-100 (-), CD31 (+), CD34 (+), ALK p80 (-), ERG (+), INI-1(+), D2-40(-), CK7(-), and Ki-67(60%+) was identified. Postoperative complications were absent, the incision exhibited optimal recovery, and the patient was discharged from the hospital within 10 days. The final diagnosis was as follows: 1. splenic malignant tumour; 2. nontraumatic splenic rupture; 3. abdominal hemoperitoneum; and 4. grade 1 hypertension.\u003c/p\u003e \u003cp\u003eOn 29 September, the patient was admitted to the oncology department of our hospital, which specializes in both traditional Chinese and Western medicine. Following a thorough examination, no obvious contraindications to chemotherapy were identified. The following day, the patient was administered 150 mg of paclitaxel liposome intravenously on days 1 and 8. A total of three and a half courses were maintained. On January 14, 2024, a CT scan revealed the presence of multiple liver tumour metastases. In comparison with the MRI slice on August 21, 2022 (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e), the patient's liver disease progression was taken into consideration, and oral Sunitinib 50 mg qd targeted therapy was initiated on January 14th.\u003c/p\u003e \u003cp\u003eOn 10 February 2024, the patient presented with acute abdominal pain, primarily in the upper abdomen and right epigastrium. He was subsequently transferred to the emergency department, where a CT scan revealed a ruptured liver with concomitant bleeding. The patient and his family convened to discuss the circumstances and considered the option of no active treatment, opting instead for automatic discharge. The patient was subsequently discharged and died shortly thereafter.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAs is well established in the literature, PSA is a lethal tumour with an unknown pathogenic mechanism, and some environmental factors (such as chemical substances such as vinyl chloride monomer, thorium dioxide, arsenic, or radiation exposure) are considered risk factors for splenic vascular sarcoma [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. One theory postulates that PSA may be the result of the malignant transformation of other benign spleen tumours, such as hemangiomas, angio endotheliomas and lymphangiomas [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. The patient in this case denied a history of prior chemical exposure or benign spleen tumours.\u003c/p\u003e \u003cp\u003eEarly diagnosis of PSA is challenging due to its low incidence and tendency to be overlooked in broad differential diagnoses. Definitive diagnosis of PSA relies on puncture biopsy or pathology, but spleen biopsy is contraindicated due to the high risk of induced rupture and metastasis and the poor diagnostic yield [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The patient had been divorced for many years, had no regular occupation, had an irregular and poor health awareness, and never attended health check-ups. On 21 August 2022, the patient underwent an MRI scan of the upper abdomen due to abdominal pain, which revealed diffuse splenic space occupation. The size of the biggest nodule was approximately 5.0*6.0 cm, and the patient was considered to have a large vasculogenic lesion, multiple cysts of the liver and hemangiomas, which were not further diagnosed at that time. On 8 August 2023, the patient was admitted to hospital with acute abdominal pain, he did not undergo an ultrasound examination, instead, a CT scan of the abdomen and pelvis was performed. This revealed a splenic rupture, and the patient was immediately taken for emergency surgery to remove the spleen. Imaging played a crucial role in preoperative diagnosis, allowing valuable time for emergency surgery. Anemia is the primary manifestation of PSA laboratory tests, with others including leukopenia, thrombocytopenia, and increased erythrocyte sedimentation rate [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In this case, the patient exhibited atypical symptoms and presented to the local health center with acute abdomen. CT revealed pelvic effusion, leading to referral to a higher-level hospital. Laboratory tests for anemia and platelet reduction up to a critical value (23*10\u003csup\u003e9\u003c/sup\u003e/L) were conducted. During the diagnostic process and subsequent treatment, routine tumour marker examinations revealed elevated neuron-specific enolase levels (18.5 ng/ml; reference range\u0026thinsp;\u0026lt;\u0026thinsp;16.3 ng/ml), with no significant anomalies observed in other indicators. The final diagnosis of this patient with PSA relied on pathological examination, and in addition to morphological features, previous studies [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e] have shown that diagnosis of this tumour requires a combination of immunohistochemical findings, and immunohistochemical testing in this patient suggested that vascular differentiation was positive for CD34 and CD31, suggesting that Vimentin, which may have originated from mesenchymal tissue, was positive, consistent with the report.\u003c/p\u003e \u003cp\u003eIn cases of PSA, given its extremely low incidence, the treatment of choice is splenectomy, which has been shown to prolong survival, both in cases of invasive disseminated metastases and in those where splenectomy is performed before splenic rupture [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Lumpectomy is not the preferred option for most physicians, given that splenomegaly increases the risk of hemorrhage, pericardium rupture, and intermediate conversion to open laparotomy. In this case, due to the occurrence of acute symptoms such as a drop in blood pressure, the surgeon considered the possibility of splenic rupture, and subsequently performed open laparotomy with splenectomy, resulting in no postoperative complications. However, there are no specific chemotherapeutic regimens for splenic angiosarcoma, and a paucity of clinical evidence from large-scale studies, with most chemotherapeutic regimens being empirically designed. Vakkalanka et al.[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] discovered that the utilisation of paclitaxel for the treatment of patients with PSA can substantially enhance their prognoses. As found by de Azevedo OS et al.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e], patients with PSA demonstrated a positive response to taxane treatment, with the treatment effectively prolonging survival time. The patient was administered a paclitaxel liposome 150 mg d1,d8 intravenous chemotherapy regimen, which was continued for three and a half courses. A review of the literature reveals that the average survival time for patients with PSA after surgery is approximately six months. The fact that the patient in this case developed a liver rupture precisely six months after surgery, and had already exhibited liver metastases prior to his demise, serves to underscore the highly aggressive nature of the disease.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003ePSA is an uncommon disease with a high degree of malignancy and an absence of clinical features, necessitating vigilance on the part of clinicians when encountering patients with long-term splenomegaly, anemia, and thrombocytopenia. In such cases, a comprehensive differential diagnosis should be sought, with surgical resection of the tumour being the optimal approach when it is still small, or at least before splenic rupture, with the aim of maximizing patient survival. Future research could concentrate on elucidating the underlying molecular mechanisms that drive the development and progression of this disease. By elucidating these mechanisms, researchers may be able to identify specific serum markers to facilitate the differential diagnosis of PSA, and to develop more targeted therapies to improve patient outcomes.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003ePSA Primary splenic angiosarcoma\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe patient was a distant relative of mine and we thank the deceased and his family for their willingness to share this case for scientific and educational purposes. We thank the medical, imaging, and pathology teams involved in the diagnosis and treatment of this rare tumour for their dedication and expertise in ensuring the best possible outcome for the patient.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWu Z.D conceived and designed the study and drafted the manuscript. Cao X.M participated in the revision of the content related to clinical diagnosis and treatment. Zuo Y.Y provided clear routine pathology and immunohistochemistry image reports. Zheng Y.Q provided clear imaging image reports. All authors read and approved the manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was funded by Jiangyin Municipal Health Commission.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical Trial Registration\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study is a retrospective case study report based on available medical data and does not involve active intervention in subjects. Consequently, clinical trial registration is not applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNo datasets were generated or analysed during the current study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical Statement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis case report was approved by the Ethics Committee of Jiangyin People\u0026apos;s Hospital in accordance with the Measures for Ethical Review of Life Science and Medical Research Involving Human Beings promulgated by the National Health and Health Commission. Written informed consent was obtained from the patient\u0026apos;s family for the publication of this report (including all clinical details and pictures). All personally identifiable information has been removed to ensure patient confidentiality.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe patient had already passed away by the time the author was considering putting the case together for publication. So written informed consent was obtained from the parents. They have consented to the submission of the case report for submission to the journal.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to publish\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent for publication of clinical details and imaging was obtained from the patient\u0026apos;s family.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability statements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll data supporting the findings of this study are available within the paper and its Supplementary Information.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003eLi, R., et al., \u003cem\u003eClinical Characteristics and Prognostic Factors of Primary Splenic Angiosarcoma: A Retrospective Clinical Analysis from China.\u003c/em\u003e Cell Physiol Biochem, 2018. 49(5): p. 1959-1969.\u003c/li\u003e\n \u003cli\u003eCao, L., et al., \u003cem\u003eA primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile.\u003c/em\u003e Medicine (Baltimore), 2019. 98(28): p. e16245.\u003c/li\u003e\n \u003cli\u003eFalk, S., J. Krishnan, and J.M. Meis, \u003cem\u003ePrimary angiosarcoma of the spleen. A clinicopathologic study of 40 cases.\u003c/em\u003e Am J Surg Pathol, 1993. 17(10): p. 959-70.\u003c/li\u003e\n \u003cli\u003eKamocki, Z., et al., \u003cem\u003ePrimary splenic angiosarcoma - the same diagnosis yielding two different clinical pictures. Case report.\u003c/em\u003e Contemp Oncol (Pozn), 2013. 17(2): p. 218-21.\u003c/li\u003e\n \u003cli\u003eBatouli, A., et al., \u003cem\u003ePrimary Splenic Angiosarcoma: Clinical and Imaging Manifestations of This Rare Aggressive Neoplasm.\u003c/em\u003e Curr Probl Diagn Radiol, 2016. 45(4): p. 284-7.\u003c/li\u003e\n \u003cli\u003eFiorentino, M.D., et al., \u003cem\u003ePrimary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum.\u003c/em\u003e Autops Case Rep, 2019. 9(3): p. e2019100.\u003c/li\u003e\n \u003cli\u003eChen, X., et al., \u003cem\u003eEarly detection and integral resection are keys to extend survival in patients suffered from primary angiosarcoma of the spleen: A care-compliant case report and literature review.\u003c/em\u003e Medicine (Baltimore), 2018. 97(5): p. e9718.\u003c/li\u003e\n \u003cli\u003eDuan, Y.F., et al., \u003cem\u003eSpontaneous rupture of primary splenic angiosarcoma: a case report and literature review.\u003c/em\u003e World J Surg Oncol, 2013. 11: p. 53.\u003c/li\u003e\n \u003cli\u003eVakkalanka, B. and M. Milhem, \u003cem\u003ePaclitaxel as neoadjuvant therapy for high grade angiosarcoma of the spleen: a brief report and literature review.\u003c/em\u003e Clin Med Insights Oncol, 2010. 4: p. 107-10.\u003c/li\u003e\n \u003cli\u003ede Azevedo, O.S., et al., \u003cem\u003eSplenic Angiosarcoma: A Diagnostic Splenectomy Finding.\u003c/em\u003e Case Rep Oncol, 2016. 9(3): p. 733-737.This case report serves as a reminder of the importance of continued research into PSA.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Splenic tumour, Angiosarcoma, Splenic rupture, Liver rupture","lastPublishedDoi":"10.21203/rs.3.rs-6348464/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6348464/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e Primary splenic angiosarcoma is an uncommon form of malignant splenic neoplasm that is characterized by rapid proliferation, significant invasiveness, and widespread metastases. However, the disease often presents with non-specific clinical manifestations, which can result in delayed diagnoses and misdiagnoses. Early diagnosis and intervention are therefore crucial for improving patient survival.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase Presentation\u003c/strong\u003e This case report provides a comprehensive account of the diagnosis and treatment of a patient with primary splenic angiosarcoma, encompassing the entire clinical trajectory of the disease, including its onset, surgical intervention, postoperative chemotherapy, and ultimately, the patient's demise due to liver metastasis and liver rupture. The report is comprehensive and meticulously detailed, thus making it a valuable reference source. Prior to the onset of the disease, the patient exhibited no specific symptoms except for a decrease in platelets. CT and MRI played an important role in the diagnosis and evaluation of the disease. Surgical removal of the spleen, in conjunction with the formulation of an empirical chemotherapy regimen, enabled the patient to survive for a period of six months following surgery. However, the patient ultimately succumbed to a liver rupture.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion \u003c/strong\u003ePrimary splenic angiosarcoma is a highly lethal condition, and prompt diagnosis and intervention are imperative for extending patients' survival. This case underscores the need for clinicians to exercise vigilance when encountering patients with prolonged thrombocytopenia, to utilize diverse diagnostic modalities to establish a differential diagnosis, and to endeavor to resect the tumour surgically while it is still small or, at the very least, before the spleen ruptures. Concurrently, there is an urgent need to develop new and more representative serum markers to facilitate differential diagnosis of the disease.\u003c/p\u003e","manuscriptTitle":"Primary splenic hemangiosarcoma with liver metastasis and liver rupture after surgery: a rare case and literature review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-09 11:14:19","doi":"10.21203/rs.3.rs-6348464/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"7700959d-9e0a-4285-93d4-9fd63c6672e8","owner":[],"postedDate":"May 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-06-05T09:09:02+00:00","versionOfRecord":[],"versionCreatedAt":"2025-05-09 11:14:19","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6348464","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6348464","identity":"rs-6348464","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}