Cutaneous Angiosarcoma in a Patient with Chronic Lymphedema: A Case Emphasizing the Importance of Early Recognition

Cutaneous Angiosarcoma in a Patient with Chronic Lymphedema: A Case Emphasizing the Importance of Early Recognition | 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 Systematic Review Cutaneous Angiosarcoma in a Patient with Chronic Lymphedema: A Case Emphasizing the Importance of Early Recognition Amina Tariq, Matthew Hemming Hemming, Minorvi Amin, Joseph Jacob, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7539048/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 We report the case of a 78-year-old female with a history of radical mastectomy for breast cancer in 2011, complicated by chronic left upper extremity (LUE) lymphedema. In September 2021, she was referred to plastic surgery for evaluation of a progressively enlarging, violaceous lesion on the LUE. Initially presumed to be a slow-healing hematoma, the lesion continued to enlarge, prompting a skin biopsy three months later that confirmed a diagnosis of cutaneous angiosarcoma. The patient declined amputation but underwent multiple local resections, chemotherapy, and radiation therapy. In June 2024, nearly two years after her initial diagnosis, she presented to the emergency department with signs of a soft tissue infection of the LUE. Imaging and laboratory workup revealed no evidence of metastatic spread. During her hospitalization, she developed worsening infection, suffered multiple strokes, and became septic. After a transition to comfort care, she passed away the same day. Angiosarcoma, a rare and aggressive subtype of soft tissue sarcoma (STS), is associated with poor prognosis and a high rate of early metastasis, with 20–40% of patients presenting with metastatic disease at diagnosis. Median survival is approximately 2.5 years with intervention, compared to 5–8 months with palliative care alone. Surgical resection remains the gold standard for localized disease. This case underscores the importance of early recognition and biopsy of suspicious lesions, particularly in patients with chronic lymphedema, to facilitate timely diagnosis and potentially improve outcomes in STS. Stewart Treves Syndrome Angiosarcoma Metastases Lymphedema Figures Figure 1 Figure 2 Figure 3 Introduction STS is a rare form of cutaneous angiosarcoma that arises in areas of chronic lymphedema. The disease was first described in 1948 by Drs. Fred Stewart and Norman Treves, after observing six female patients that developed angiosarcoma following radical mastectomy (X). Although this syndrome is known to arise as a consequence of mastectomy, it can also occur in the setting of chronic infection, chronic venous stasis, morbid obesity, and malignant obstruction (II). The classic presentation consists of enlarging purple papules arising in lymphedematous areas that eventually infiltrate tissue and lead to edema, ulcerations, infections and hemorrhage (I). We report a case of a 78-year-old female who was diagnosed with angiosarcoma ten years after radical mastectomy for breast cancer complicated by chronic lymphedema consistent with STS. Case Report The patient was a 78-year-old, Caucasian female with an extensive oncologic history including colonic carcinoid tumor resected in 2002, ER+, PR+, HER stage 2 carcinoma of the left breast for which she underwent breast lumpectomy, adjuvant chemotherapy and radiation complicated by lymphedema that was managed with a lymphedema sleeve. Her oncologic history is further notable for a duodenal neuroendocrine tumor with regional nodal metastases diagnosed in 2019, endometrial adenocarcinoma of the uterus diagnosed in 2020 followed by intrauterine device placement and cessation of hormone therapy for breast cancer, and intraductal papillary mucinous neoplasm of the pancreas. Despite her multiple cancer diagnoses, germline testing did not find a cancer predisposition syndrome. Other medical problems included obesity, glaucoma, obstructive sleep apnea, hypothyroidism, and hypertension. Notably, the patient also had a history of prior taxane exposure with persistent neuropathy. In September 2022, the patient presented to her primary care physician for subcutaneous lumps with color change on her left upper extremity for the past three months, prompting a referral to plastic surgery for potential biopsy. She saw plastic surgery in October 2022, who observed “bruising/hardening of the superficial subcutaneous tissue [on the posterior arm] without underlying palpable mass or fluid” and diagnosed a slow-healing bruise. In November 2022, the patient visited her hematologist with concerns that the lesion was now painful and larger than 10 centimeters. Given no history of easy bruising and an adequate platelet count (110,000) with normal INR, there was low suspicion for coagulopathy. A punch biopsy of the left upper arm, below the area of the lesion, was performed and revealed normal skin (see Fig. 1). An MRI was then ordered by her oncologist, with notable findings including nodular and mass-like skin thickening at the dorsal ulnar aspect of the upper arm. By December, the lesion had continued to progress, so an excisional biopsy of the lesion was obtained, with pathology confirming high-grade angiosarcoma involving deep and peripheral margins with vascular invasion. The patient underwent two local resections in 2022 and 2023, completed eleven cycles of liposomal doxorubicin and three courses of radiation therapy, but consistently declined amputation as a definitive surgical treatment. The patient was longitudinally treated by a wound care clinic due to drainage from the superficial lesions. Timeline of Treatment of STS: In June 2024, the patient presented to the emergency room with an infection of the left upper extremity. The option of amputation was again presented and refused by the patient. She received two weeks of empiric antibiotic treatment with overall improvement. However, the patient re-presented two weeks later with worsening drainage and was found to have multiple metabolic derangements in the setting of gram-negative bacteremia. Despite treatment with broad spectrum intravenous antibiotics, the patient continued to decline and developed multiple acute strokes. Ultimately, the decision was made to transition her to comfort care and she died a few hours later. Discussion STS is a rare form of angiosarcoma with poor prognosis that arises in patients with chronic lymphedema. Although most documented cases develop at sites of chronic lymphedema after radical surgery for breast cancer, it may arise in other forms of congenital and acquired lymphedema such as morbid obesity, venous stasis, and surgical trauma (III). Presentation STS presents with progressing red and purple ecchymoses which can lead to pain and bleeding in the later stages. Sometimes, STS can present as a violaceous papule that can be mistaken as a hematoma, as in our patient’s case. However, these lesions rapidly progress to ulcerating purple-red papules that can necrotize and bleed. There may also be atrophic skin, hyperkeratosis, and telangiectasias associated with the classical dermatological findings of STS (IV). A key differential diagnosis for this syndrome is radiation induced angiosarcoma. The key finding differentiation this from STS is that in STS, there is a key clinical finding of chronic lymphedema without radiation whereas in the radiation induced angiosarcoma, the patient only has radiation exposure without any edema (XX). Clinically, it is very difficult to diagnose the difference as they present the same way. However, using the clinical history and timeline can help differentiate between the conditions. Diagnostic Testing The diagnosis of STS is a clinical one supported with imaging and immunohistochemical findings. Histological findings usually include proliferating vascular channels lined by tumor cells undergoing mitotic changes and displaying hyperchromatism and pleomorphism. The immunohistochemical stains for angiosarcoma include MYC, CD31, CD34, Factor VII, FL-1, ERG (II). In addition to these tests, the medical history and nature of the lesions should be taken into account. Our patient’s progressive skin lesion was initially mistaken for a hematoma. In retrospect, the lesion, in addition to its location (upper extremity), lack of preceding trauma, underlying lymphedema, and prior history of chemotherapy, radiation, and radical mastectomy for breast cancer, raised suspicion for STS. It is important for physicians to be cautious of lesions and skin discolorations in patients who have had a history of chronic lymphedema and mastectomy or radiation Treatment Standard treatment for STS is a radical surgical approach due to the aggressive nature of the cancer. The mainstay treatment with the greatest chance of survival is radical amputation of the area of angiosarcoma to minimize the extent of metastatic disease and infection (VI). The five-year survival rate for those who undergo radical amputation is 80%. Patients can also choose to take a conservative approach with chemotherapy and radiation, although this approach is associated with a shorter survival of 15 to 18 months (XII). Many chemotherapy agents such as pembrolizumab, paclitaxel, doxorubicin, gemcitabine, and ifosfamide/anthracycline can be used therapeutically to manage STS, however, overall survival is limited to 8–31 months (XV, XIII, XVII). Summary of Outcomes from Case Reports with Varying Treatments for STS with Upper Limb Edema Case Study (reference) Treatment Approach Follow Up Metastasis 1 (X) TNF- alpha Inhibitor Died 10 months yes 2 (V) ifosfamide Died in 4 months yes 3 (XXII) doxorubicin Died in 18 months yes 4 (X) Extended resection + amputation Survived for 72 months no 5 (X) Extended resection chemotherapy Died in 31 months no 6 (X) 4 patients with Forequarter amputation Disease free for 3,16, 135 months no Prognosis Despite the various treatment options, the overall prognosis of STS is poor due to the high rate of recurrence and metastatic disease. Some common areas of metastasis include the lungs, thoracic wall, liver, bones, soft tissue, brain and lymph nodes. With the frequently delayed diagnosis of STS, approximately 20–40% of patients have metastasis at the time of diagnosis. Metastatic disease at the time of diagnosis plays a significant role in its prognosis (VII). The five-year survival rate for localized angiosarcoma ranges from 30–40% compared to 10–15% for metastatic angiosarcoma (XXI), thus, early diagnosis is critical and can positively influence survival rate. (XIX). Earlier diagnosis may facilitate complete surgical resection. With later diagnosis or metastatic spread of tumor, complete surgical resection is often not feasible, leading to recurrence as seen in our patient, and metastasis (IX). Our patient never developed detectable distant metastases, but despite undergoing two local resections, she did develop local recurrence throughout the LUE. Infection is another complication seen in STS (VIII). Our patient developed a life-threatening infection of the LUE requiring hospitalization twice within the course of one month that ultimately triggered other sepsis-related complications which she died from. This case demonstrates the outcome of STS in a patient with a delay in diagnosis, and who chose non-surgical management throughout the disease course. The case highlights the importance of clinicians to understand the clinical presentation and course of STS in order to diagnose it promptly. Early diagnosis can mitigate tumor progression and metastasis. STS must be part of the differential diagnosis in any patient with a history of chronic lymphedema who presents with progressively worsening red-purple raised papules. Given the rapid progression and poor prognosis, it is also important to discuss the risks and benefits of the different treatment options at initial diagnosis. We hope that this case continues to draw attention to this rare disease and offer guidance to physicians who come across these patients in practice. Declarations Conflict of Interest: None Consent The authors obtained written consent from patients for their photographs and medical information to be published in print and online and with the understanding that this information may be publicly available. Patient consent forms were not provided to the journal but are retained by the authors Funding: none Author Contribution A.T: Wrote the main manuscript text M.H and M.A edited and revised the main manuscript J.J provided images and description for CT and MRI imagingE.M and A.A provided appropriate image and description for histopathology Acknowledgements: I would like to acknowledge my patient and her family for giving us the opportunity to learn from her. I would also like to thank Dr. Hemming and Dr. Amin for their unwavering support. References Sharma A, Schwartz RA (2012) Stewart-Treves syndrome: Pathogenesis and management. J Am Acad Dermatol 67(6):1342–1348. 10.1016/j.jaad.2012.04.028 Gottlieb R, Serang R, Chi D, Menco H (2015) Stewart-Treves syndrome. Radiol Case Rep 7(4):693. 10.2484/rcr.v7i4.693 PMID: 27330592; PMCID: PMC4899547 Murgia RD, Gross GP, Stewart-Treves Syndrome [Updated 2023 Aug 14]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/ Armstrong J, Cardwell G, Brinker A (2022) Stewart-Treves syndrome of the lower extremity: case report. AME Med J [Online]. ;7. Available from: https://journals.amegroups.com/amej/article/view/82825 Vojtíšek R, Sukovská E, Kylarová M, Kacerovská D, Baxa J, Divišová B, Fínek J (2020 Nov-Dec) Stewart-Treves syndrome: Case report and literature review. Rep Pract Oncol Radiother 25(6):934–938. 10.1016/j.rpor.2020.09.006 Epub 2020 Oct 1. PMID: 33088229; PMCID: PMC7550831 A rare case (2007) of post-mastectomy lymphangiosarcoma. J Lymphoedema 2(1):1–6 O'Donnell TF, Allison GM, Melikian R, Iafrati MD (2020) A systematic review of the quality of clinical practice guidelines for lymphedema, as assessed using the Appraisal of Guidelines for Research and Evaluation II instrument. J Vasc Surg Venous Lymphat Disord 8(4):685–692. 10.1016/j.jvsv.2020.04.008 Murgia RD, Gross GP, Stewart-Treves Syndrome [Updated 2023 Aug 14]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/ Schiffman S, Berger A (2007) Stewart-Treves syndrome. J Am Coll Surg . ;204(2):328. 10.1016/j.jamcollsurg.2006.06.033 . Epub 2006 Sep 26. PMID: 17254938 Murgia RD, Gross GP, Stewart-Treves S (2023) Aug 14. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. PMID: 29939610 Mallon E, Powell S, Mortimer P et al (1997) Evidence for altered cell-mediated immunity in postmastectomy lymphoedema. Br J Dermatol . ;137:928–933. Available from: https://pmc.ncbi.nlm.nih.gov/articles/PMC4836142/ Pereira ES, Moraes ET, Siqueira DM, Santos MA (2015) Stewart Treves Syndrome. An Bras Dermatol . May-Jun;90(3 Suppl 1):229–231. 10.1590/abd1806-4841.20153685 . PMID: 26312725; PMCID: PMC4540559 Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ, Angiosarcoma (2010) Lancet Oncol 11(10):983–991 National Comprehensive Cancer Network Soft Tissue Sarcoma . Version 2.2023. Available from: https://www.nccn.org/professionals/physician_gls/pdf/sarcoma_blocks.pdf Diagnostic Pathology. Stewart-Treves Syndrome: Case Review. Diagn Pathol (2023) Available from: https://diagnosticpathology.biomedcentral.com/articles/ 10.1186/s13000-023-01365-1 American Cancer Society Survival rates for soft tissue sarcoma. [Cited 2024 Oct 10]. Available from: https://www.cancer.org/cancer/types/soft-tissue-sarcoma/detection-diagnosis-staging/survival-rates.html Kokkali S, Moreno JD, Klijanienko J, Theocharis S (2022) Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease? Int J Mol Sci 23(8):4125. 10.3390/ijms23084125 PMID: 35456944; PMCID: PMC9029574 Madan R, Kaffenberger BH, Deneve JL et al (2010) Stewart-Treves syndrome: A review and analysis of 160 cases. J Am Acad Dermatol 62(3):396–404 McKeown DG, Boland PJ (2013) Stewart-Treves syndrome: A case report. Ann R Coll Surg Engl 95(5):e6–e8 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. 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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-7539048","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Systematic Review","associatedPublications":[],"authors":[{"id":513362980,"identity":"022d1382-a738-4771-a340-bae14abaa17e","order_by":0,"name":"Amina Tariq","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABA0lEQVRIiWNgGAWjYFACxgYIzczA+AAmJgHiEtBiAFLDbECkFjAAK2aTIEoL/7TDbQ9+MPyRN29n3lbNm2NnLz/t8MEbDBXWiQ04tEjcTmw37GEwMJxzmK3sNu+25MQNt9OSLRjOpOPUwnA7sU2Ch8GAcQYzjxlQC3OCgXSOmQRj22GcWuSBWiT/MBjYg7QU826rt5efnf9NgvEfbi0GQC3SQFsSQVqYebcdZmy4ncMmwdiAW4shSIuMgXHyDGa2Ysm5246D/GJskXAs3RiXFrnb6c8k31TI2c7gP7zxw9tt1UCHJT+88aHGWhan9yHOQyLBIAGvcnSNo2AUjIJRMArQAQAt9VJE73sqwQAAAABJRU5ErkJggg==","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":true,"prefix":"","firstName":"Amina","middleName":"","lastName":"Tariq","suffix":""},{"id":513362981,"identity":"ba110f43-d982-4999-9067-8c1eef9825ea","order_by":1,"name":"Matthew Hemming Hemming","email":"","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":false,"prefix":"","firstName":"Matthew","middleName":"Hemming","lastName":"Hemming","suffix":""},{"id":513362982,"identity":"43dbd92e-672d-4804-8f09-d5b813cee7e2","order_by":2,"name":"Minorvi Amin","email":"","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":false,"prefix":"","firstName":"Minorvi","middleName":"","lastName":"Amin","suffix":""},{"id":513362983,"identity":"2948c4eb-4e5c-4bf9-a565-4f451d134b4a","order_by":3,"name":"Joseph Jacob","email":"","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":false,"prefix":"","firstName":"Joseph","middleName":"","lastName":"Jacob","suffix":""},{"id":513362984,"identity":"a77e844c-c5ff-40e3-b6d7-3eb5841c3045","order_by":4,"name":"Eric Magnetta","email":"","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":false,"prefix":"","firstName":"Eric","middleName":"","lastName":"Magnetta","suffix":""},{"id":513362985,"identity":"d9adc36f-58d2-4251-85cf-73b4a8f234c3","order_by":5,"name":"Ali Akalin","email":"","orcid":"","institution":"University of Massachusetts Chan Medical School","correspondingAuthor":false,"prefix":"","firstName":"Ali","middleName":"","lastName":"Akalin","suffix":""}],"badges":[],"createdAt":"2025-09-04 20:38:15","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7539048/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7539048/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":91199060,"identity":"238ff447-90a5-45f7-b131-5b4fdffea658","added_by":"auto","created_at":"2025-09-12 15:18:30","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":113816,"visible":true,"origin":"","legend":"\u003cp\u003ePicture of Lesion Progression from 10/2022-1/26/2023\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7539048/v1/bde997548c55a5d40f0dde68.jpg"},{"id":91200481,"identity":"57cfd9b6-fede-454c-86eb-55e013aa9e64","added_by":"auto","created_at":"2025-09-12 15:26:30","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":403369,"visible":true,"origin":"","legend":"\u003cp\u003ePATHOLOGY\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eInitial punch biopsy:\u003c/em\u003e “normal skin with minimal superficial lymphohistiocytic inflammation with mild mucin. Prussian blue was negative for iron.”\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eExcisional biopsy Report: “\u003c/em\u003eExcisional Biopsy revealed CD31+, CD34+, ERG+, Pan CK+, and CK7+, negative for cytokeratin, OSCAR, chromogranin, synaptophysin, CDX2, PAX8, CD34, and GATA3. She has extensive lobular/tumor vascular proliferation with high grade cytological atypia, epithelioid as well as spindle cell morphology, involving the entire dermis and the subcutaneous fat. Tumor invasion into a large blood vessel is also identified. Tissue margins, including the deep margin, are invaded by the tumor extensively. This confirms the diagnosis of angiosarcoma.”\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7539048/v1/cb2f8840af696204acf894ee.jpg"},{"id":91199062,"identity":"4cb95908-d528-47ff-8a2c-3e622776511d","added_by":"auto","created_at":"2025-09-12 15:18:30","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":171551,"visible":true,"origin":"","legend":"\u003cp\u003eIMAGING\u003c/p\u003e\n\u003cp\u003e\u003cem\u003e\u003cstrong\u003eDescription Figure III A,B,C: \u003c/strong\u003e\u003c/em\u003e\u003cem\u003eMRI of the left arm with and without contrast from 1/7/2023\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eMagnetic resonance imaging (MRI) performed after the initial incision and drainage is shown above. Axial T1 (no fat suppression) (image 1), axial T1 post-contrast with fat suppression (image 2), and sagittal STIR (image 3) images demonstrate an ill-defined, mildly exophytic, nodular mass centered within the skin and extending into the subcutaneous soft tissues of the dorsal, ulnar aspect of the distal upper arm. There is adjacent skin thickening and subcutaneous edema. The depth/thickness of the mass has increased from 1.3 cm on the prior motion-degraded, prematurely-terminated MRI from 1 month ago, to 3.0 cm. The mass is heterogeneously T2 hyperintense and predominately T1 isointense to slightly hyperintense to muscle. The mass likely demonstrates heterogeneous nodular enhancement.\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDescription Figure III D,E,F,G,H:\u003c/strong\u003e Initial fluorodeoxyglucose (FDG) PET/CT from 1/13/2023\u003cbr\u003e\n Fusion FDG PET/CT image (figure IIId) and the corresponding noncontrast CT in soft tissue window (figure IIIe) demonstrates an irregular, nodular, intensely FDG-avid cutaneous/subcutaneous mass within the distal left upper arm [corresponding to the primary mass seen on MRI]. Fusion FDG PET/CT (figure IIIf) and noncontrast CT in soft tissue window (figure IIIg) depict an adjacent separate intensely FDG-avid satellite nodule inferolateral to the dominant lesion. Maximum intensity projection (MIP) PET image (figure IIIh) shows both the dominant and satellite lesion within the left upper arm. [Both lesions demonstrate intense FDG uptake, causing them to blend into one another on the PET MIP.]​\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7539048/v1/31b9f2cd311f37410dbfdb41.jpg"},{"id":91200808,"identity":"65dfce74-939a-42ef-80d6-65a9f8158449","added_by":"auto","created_at":"2025-09-12 15:34:30","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1168834,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7539048/v1/ccf00cf4-b145-4db2-a3ba-e634745e41ca.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Cutaneous Angiosarcoma in a Patient with Chronic Lymphedema: A Case Emphasizing the Importance of Early Recognition","fulltext":[{"header":"Introduction","content":"\u003cp\u003eSTS is a rare form of cutaneous angiosarcoma that arises in areas of chronic lymphedema. The disease was first described in 1948 by Drs. Fred Stewart and Norman Treves, after observing six female patients that developed angiosarcoma following radical mastectomy (X). Although this syndrome is known to arise as a consequence of mastectomy, it can also occur in the setting of chronic infection, chronic venous stasis, morbid obesity, and malignant obstruction (II). The classic presentation consists of enlarging purple papules arising in lymphedematous areas that eventually infiltrate tissue and lead to edema, ulcerations, infections and hemorrhage (I).\u003c/p\u003e\u003cp\u003eWe report a case of a 78-year-old female who was diagnosed with angiosarcoma ten years after radical mastectomy for breast cancer complicated by chronic lymphedema consistent with STS.\u003c/p\u003e"},{"header":"Case Report","content":"\u003cp\u003eThe patient was a 78-year-old, Caucasian female with an extensive oncologic history including colonic carcinoid tumor resected in 2002, ER+, PR+, HER stage 2 carcinoma of the left breast for which she underwent breast lumpectomy, adjuvant chemotherapy and radiation complicated by lymphedema that was managed with a lymphedema sleeve. Her oncologic history is further notable for a duodenal neuroendocrine tumor with regional nodal metastases diagnosed in 2019, endometrial adenocarcinoma of the uterus diagnosed in 2020 followed by intrauterine device placement and cessation of hormone therapy for breast cancer, and intraductal papillary mucinous neoplasm of the pancreas. Despite her multiple cancer diagnoses, germline testing did not find a cancer predisposition syndrome. Other medical problems included obesity, glaucoma, obstructive sleep apnea, hypothyroidism, and hypertension. Notably, the patient also had a history of prior taxane exposure with persistent neuropathy.\u003c/p\u003e\u003cp\u003eIn September 2022, the patient presented to her primary care physician for subcutaneous lumps with color change on her left upper extremity for the past three months, prompting a referral to plastic surgery for potential biopsy. She saw plastic surgery in October 2022, who observed \u0026ldquo;bruising/hardening of the superficial subcutaneous tissue [on the posterior arm] without underlying palpable mass or fluid\u0026rdquo; and diagnosed a slow-healing bruise. In November 2022, the patient visited her hematologist with concerns that the lesion was now painful and larger than 10 centimeters. Given no history of easy bruising and an adequate platelet count (110,000) with normal INR, there was low suspicion for coagulopathy. A punch biopsy of the left upper arm, below the area of the lesion, was performed and revealed normal skin (see Fig.\u0026nbsp;1). An MRI was then ordered by her oncologist, with notable findings including nodular and mass-like skin thickening at the dorsal ulnar aspect of the upper arm. By December, the lesion had continued to progress, so an excisional biopsy of the lesion was obtained, with pathology confirming high-grade angiosarcoma involving deep and peripheral margins with vascular invasion. The patient underwent two local resections in 2022 and 2023, completed eleven cycles of liposomal doxorubicin and three courses of radiation therapy, but consistently declined amputation as a definitive surgical treatment. The patient was longitudinally treated by a wound care clinic due to drainage from the superficial lesions.\u003c/p\u003e\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eTimeline of Treatment of STS: \u003c/h2\u003e\u003cp\u003eIn June 2024, the patient presented to the emergency room with an infection of the left upper extremity. The option of amputation was again presented and refused by the patient. She received two weeks of empiric antibiotic treatment with overall improvement. However, the patient re-presented two weeks later with worsening drainage and was found to have multiple metabolic derangements in the setting of gram-negative bacteremia. Despite treatment with broad spectrum intravenous antibiotics, the patient continued to decline and developed multiple acute strokes. Ultimately, the decision was made to transition her to comfort care and she died a few hours later.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eSTS is a rare form of angiosarcoma with poor prognosis that arises in patients with chronic lymphedema. Although most documented cases develop at sites of chronic lymphedema after radical surgery for breast cancer, it may arise in other forms of congenital and acquired lymphedema such as morbid obesity, venous stasis, and surgical trauma (III).\u003c/p\u003e\u003cp\u003e\u003cstrong\u003ePresentation\u003c/strong\u003e\u003cp\u003eSTS presents with progressing red and purple ecchymoses which can lead to pain and bleeding in the later stages. Sometimes, STS can present as a violaceous papule that can be mistaken as a hematoma, as in our patient\u0026rsquo;s case. However, these lesions rapidly progress to ulcerating purple-red papules that can necrotize and bleed. There may also be atrophic skin, hyperkeratosis, and telangiectasias associated with the classical dermatological findings of STS (IV).\u003c/p\u003e\u003c/p\u003e\u003cp\u003eA key differential diagnosis for this syndrome is radiation induced angiosarcoma. The key finding differentiation this from STS is that in STS, there is a key clinical finding of chronic lymphedema without radiation whereas in the radiation induced angiosarcoma, the patient only has radiation exposure without any edema (XX). Clinically, it is very difficult to diagnose the difference as they present the same way. However, using the clinical history and timeline can help differentiate between the conditions.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eDiagnostic Testing\u003c/strong\u003e\u003cp\u003eThe diagnosis of STS is a clinical one supported with imaging and immunohistochemical findings.\u003c/p\u003e\u003c/p\u003e\u003cp\u003eHistological findings usually include proliferating vascular channels lined by tumor cells undergoing mitotic changes and displaying hyperchromatism and pleomorphism. The immunohistochemical stains for angiosarcoma include MYC, CD31, CD34, Factor VII, FL-1, ERG (II).\u003c/p\u003e\u003cp\u003eIn addition to these tests, the medical history and nature of the lesions should be taken into account. Our patient\u0026rsquo;s progressive skin lesion was initially mistaken for a hematoma. In retrospect, the lesion, in addition to its location (upper extremity), lack of preceding trauma, underlying lymphedema, and prior history of chemotherapy, radiation, and radical mastectomy for breast cancer, raised suspicion for STS. It is important for physicians to be cautious of lesions and skin discolorations in patients who have had a history of chronic lymphedema and mastectomy or radiation\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eTreatment\u003c/strong\u003e\u003cp\u003eStandard treatment for STS is a radical surgical approach due to the aggressive nature of the cancer. The mainstay treatment with the greatest chance of survival is radical amputation of the area of angiosarcoma to minimize the extent of metastatic disease and infection (VI). The five-year survival rate for those who undergo radical amputation is 80%. Patients can also choose to take a conservative approach with chemotherapy and radiation, although this approach is associated with a shorter survival of 15 to 18 months (XII). Many chemotherapy agents such as pembrolizumab, paclitaxel, doxorubicin, gemcitabine, and ifosfamide/anthracycline can be used therapeutically to manage STS, however, overall survival is limited to 8\u0026ndash;31 months (XV, XIII, XVII).\u003c/p\u003e\u003c/p\u003e\n\u003ch3\u003eSummary of Outcomes from Case Reports with Varying Treatments for STS with Upper Limb Edema\u003c/h3\u003e\n\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"No\" id=\"Taba\" border=\"1\"\u003e\u003ccolgroup cols=\"4\"\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\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eCase Study \u003csup\u003e(reference)\u003c/sup\u003e\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTreatment Approach\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eFollow Up\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eMetastasis\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e1\u003csup\u003e(X)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTNF- alpha Inhibitor\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDied 10 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eyes\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e2\u003csup\u003e(V)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eifosfamide\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDied in 4 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eyes\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e3\u003csup\u003e(XXII)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003edoxorubicin\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDied in 18 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eyes\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e4\u003csup\u003e(X)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eExtended resection\u0026thinsp;+\u0026thinsp;amputation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eSurvived for 72 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eno\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e5\u003csup\u003e(X)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eExtended resection chemotherapy\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDied in 31 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eno\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e6\u003csup\u003e(X)\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e4 patients with Forequarter amputation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDisease free for 3,16, 135 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eno\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\u003e\u003cstrong\u003ePrognosis\u003c/strong\u003e\u003cp\u003eDespite the various treatment options, the overall prognosis of STS is poor due to the high rate of recurrence and metastatic disease. Some common areas of metastasis include the lungs, thoracic wall, liver, bones, soft tissue, brain and lymph nodes. With the frequently delayed diagnosis of STS, approximately 20\u0026ndash;40% of patients have metastasis at the time of diagnosis. Metastatic disease at the time of diagnosis plays a significant role in its prognosis (VII). The five-year survival rate for localized angiosarcoma ranges from 30\u0026ndash;40% compared to 10\u0026ndash;15% for metastatic angiosarcoma (XXI), thus, early diagnosis is critical and can positively influence survival rate. (XIX). Earlier diagnosis may facilitate complete surgical resection. With later diagnosis or metastatic spread of tumor, complete surgical resection is often not feasible, leading to recurrence as seen in our patient, and metastasis (IX). Our patient never developed detectable distant metastases, but despite undergoing two local resections, she did develop local recurrence throughout the LUE. Infection is another complication seen in STS (VIII). Our patient developed a life-threatening infection of the LUE requiring hospitalization twice within the course of one month that ultimately triggered other sepsis-related complications which she died from.\u003c/p\u003e\u003c/p\u003e\u003cp\u003eThis case demonstrates the outcome of STS in a patient with a delay in diagnosis, and who chose non-surgical management throughout the disease course. The case highlights the importance of \u003cb\u003eclinicians to understand\u003c/b\u003e the clinical presentation and course of STS in order to diagnose it promptly. Early diagnosis can mitigate tumor progression and metastasis. STS must be part of the differential diagnosis in any patient with a history of chronic lymphedema who presents with progressively worsening red-purple raised papules. Given the rapid progression and poor prognosis, it is also important to discuss the risks and benefits of the different treatment options at initial diagnosis. We hope that this case continues to draw attention to this rare disease and offer guidance to physicians who come across these patients in practice.\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eConflict of Interest:\u003c/h2\u003e\u003cp\u003eNone\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConsent\u003c/strong\u003e\u003cp\u003e The authors obtained written consent from patients for their photographs and medical information to be published in print and online and with the understanding that this information may be publicly available. Patient consent forms were not provided to the journal but are retained by the authors\u003c/p\u003e\u003c/p\u003e\u003ch2\u003eFunding:\u003c/h2\u003e\u003cp\u003enone\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eA.T: Wrote the main manuscript text M.H and M.A edited and revised the main manuscript J.J provided images and description for CT and MRI imagingE.M and A.A provided appropriate image and description for histopathology\u003c/p\u003e\u003ch2\u003eAcknowledgements:\u003c/h2\u003e\u003cp\u003eI would like to acknowledge my patient and her family for giving us the opportunity to learn from her. I would also like to thank Dr. Hemming and Dr. Amin for their unwavering support.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eSharma A, Schwartz RA (2012) Stewart-Treves syndrome: Pathogenesis and management. J Am Acad Dermatol 67(6):1342\u0026ndash;1348. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jaad.2012.04.028\u003c/span\u003e\u003cspan address=\"10.1016/j.jaad.2012.04.028\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eGottlieb R, Serang R, Chi D, Menco H (2015) Stewart-Treves syndrome. Radiol Case Rep 7(4):693. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.2484/rcr.v7i4.693\u003c/span\u003e\u003cspan address=\"10.2484/rcr.v7i4.693\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 27330592; PMCID: PMC4899547\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMurgia RD, Gross GP, Stewart-Treves Syndrome [Updated 2023 Aug 14]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.ncbi.nlm.nih.gov/books/NBK507833/\u003c/span\u003e\u003cspan address=\"https://www.ncbi.nlm.nih.gov/books/NBK507833/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eArmstrong J, Cardwell G, Brinker A (2022) Stewart-Treves syndrome of the lower extremity: case report. \u003cem\u003eAME Med J\u003c/em\u003e[Online]. ;7. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://journals.amegroups.com/amej/article/view/82825\u003c/span\u003e\u003cspan address=\"https://journals.amegroups.com/amej/article/view/82825\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eVojt\u0026iacute;šek R, Sukovsk\u0026aacute; E, Kylarov\u0026aacute; M, Kacerovsk\u0026aacute; D, Baxa J, Divišov\u0026aacute; B, F\u0026iacute;nek J (2020 Nov-Dec) Stewart-Treves syndrome: Case report and literature review. Rep Pract Oncol Radiother 25(6):934\u0026ndash;938. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.rpor.2020.09.006\u003c/span\u003e\u003cspan address=\"10.1016/j.rpor.2020.09.006\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003eEpub 2020 Oct 1. PMID: 33088229; PMCID: PMC7550831\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eA rare case (2007) of post-mastectomy lymphangiosarcoma. J Lymphoedema 2(1):1\u0026ndash;6\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eO'Donnell TF, Allison GM, Melikian R, Iafrati MD (2020) A systematic review of the quality of clinical practice guidelines for lymphedema, as assessed using the Appraisal of Guidelines for Research and Evaluation II instrument. J Vasc Surg Venous Lymphat Disord 8(4):685\u0026ndash;692. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jvsv.2020.04.008\u003c/span\u003e\u003cspan address=\"10.1016/j.jvsv.2020.04.008\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMurgia RD, Gross GP, Stewart-Treves Syndrome [Updated 2023 Aug 14]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.ncbi.nlm.nih.gov/books/NBK507833/\u003c/span\u003e\u003cspan address=\"https://www.ncbi.nlm.nih.gov/books/NBK507833/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSchiffman S, Berger A (2007) Stewart-Treves syndrome. \u003cem\u003eJ Am Coll Surg\u003c/em\u003e. ;204(2):328. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jamcollsurg.2006.06.033\u003c/span\u003e\u003cspan address=\"10.1016/j.jamcollsurg.2006.06.033\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. Epub 2006 Sep 26. PMID: 17254938\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMurgia RD, Gross GP, Stewart-Treves S (2023) Aug 14. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. PMID: 29939610\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMallon E, Powell S, Mortimer P et al (1997) Evidence for altered cell-mediated immunity in postmastectomy lymphoedema. \u003cem\u003eBr J Dermatol\u003c/em\u003e. ;137:928\u0026ndash;933. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://pmc.ncbi.nlm.nih.gov/articles/PMC4836142/\u003c/span\u003e\u003cspan address=\"https://pmc.ncbi.nlm.nih.gov/articles/PMC4836142/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003ePereira ES, Moraes ET, Siqueira DM, Santos MA (2015) Stewart Treves Syndrome. \u003cem\u003eAn Bras Dermatol\u003c/em\u003e. May-Jun;90(3 Suppl 1):229\u0026ndash;231. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1590/abd1806-4841.20153685\u003c/span\u003e\u003cspan address=\"10.1590/abd1806-4841.20153685\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. PMID: 26312725; PMCID: PMC4540559\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eYoung RJ, Brown NJ, Reed MW, Hughes D, Woll PJ, Angiosarcoma (2010) Lancet Oncol 11(10):983\u0026ndash;991\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eNational Comprehensive Cancer Network \u003cem\u003eSoft Tissue Sarcoma\u003c/em\u003e. Version 2.2023. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.nccn.org/professionals/physician_gls/pdf/sarcoma_blocks.pdf\u003c/span\u003e\u003cspan address=\"https://www.nccn.org/professionals/physician_gls/pdf/sarcoma_blocks.pdf\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDiagnostic Pathology. Stewart-Treves Syndrome: Case Review. Diagn Pathol (2023) Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://diagnosticpathology.biomedcentral.com/articles/\u003c/span\u003e\u003cspan address=\"https://diagnosticpathology.biomedcentral.com/articles/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1186/s13000-023-01365-1\u003c/span\u003e\u003cspan address=\"10.1186/s13000-023-01365-1\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAmerican Cancer Society Survival rates for soft tissue sarcoma. [Cited 2024 Oct 10]. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.cancer.org/cancer/types/soft-tissue-sarcoma/detection-diagnosis-staging/survival-rates.html\u003c/span\u003e\u003cspan address=\"https://www.cancer.org/cancer/types/soft-tissue-sarcoma/detection-diagnosis-staging/survival-rates.html\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKokkali S, Moreno JD, Klijanienko J, Theocharis S (2022) Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease? Int J Mol Sci 23(8):4125. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/ijms23084125\u003c/span\u003e\u003cspan address=\"10.3390/ijms23084125\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 35456944; PMCID: PMC9029574\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMadan R, Kaffenberger BH, Deneve JL et al (2010) Stewart-Treves syndrome: A review and analysis of 160 cases. J Am Acad Dermatol 62(3):396\u0026ndash;404\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMcKeown DG, Boland PJ (2013) Stewart-Treves syndrome: A case report. Ann R Coll Surg Engl 95(5):e6\u0026ndash;e8\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"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":"Stewart Treves Syndrome, Angiosarcoma, Metastases, Lymphedema","lastPublishedDoi":"10.21203/rs.3.rs-7539048/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7539048/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eWe report the case of a 78-year-old female with a history of radical mastectomy for breast cancer in 2011, complicated by chronic left upper extremity (LUE) lymphedema. In September 2021, she was referred to plastic surgery for evaluation of a progressively enlarging, violaceous lesion on the LUE. Initially presumed to be a slow-healing hematoma, the lesion continued to enlarge, prompting a skin biopsy three months later that confirmed a diagnosis of cutaneous angiosarcoma.\u003c/p\u003e\u003cp\u003eThe patient declined amputation but underwent multiple local resections, chemotherapy, and radiation therapy. In June 2024, nearly two years after her initial diagnosis, she presented to the emergency department with signs of a soft tissue infection of the LUE. Imaging and laboratory workup revealed no evidence of metastatic spread. During her hospitalization, she developed worsening infection, suffered multiple strokes, and became septic. After a transition to comfort care, she passed away the same day.\u003c/p\u003e\u003cp\u003eAngiosarcoma, a rare and aggressive subtype of soft tissue sarcoma (STS), is associated with poor prognosis and a high rate of early metastasis, with 20\u0026ndash;40% of patients presenting with metastatic disease at diagnosis. Median survival is approximately 2.5 years with intervention, compared to 5\u0026ndash;8 months with palliative care alone. Surgical resection remains the gold standard for localized disease. This case underscores the importance of early recognition and biopsy of suspicious lesions, particularly in patients with chronic lymphedema, to facilitate timely diagnosis and potentially improve outcomes in STS.\u003c/p\u003e","manuscriptTitle":"Cutaneous Angiosarcoma in a Patient with Chronic Lymphedema: A Case Emphasizing the Importance of Early Recognition","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-09-12 15:18:25","doi":"10.21203/rs.3.rs-7539048/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":"ffe86587-a6e6-484a-83d4-d6a2cba9294a","owner":[],"postedDate":"September 12th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-09-12T15:18:25+00:00","versionOfRecord":[],"versionCreatedAt":"2025-09-12 15:18:25","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7539048","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7539048","identity":"rs-7539048","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}