Autopsy and Case Reports
Autopsy and Case Reports
Article / Clinical Case Report

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

Matheus Dalben Fiorentino; Jean Michel Correia Monteiro; Renata Elisie Barbalho de Siqueira; Elizabeth Im Myung Kim; Ana Paula Curi; Cristiane Rubia Ferrreira; Mirella Nardo; Fernando Peixoto Ferraz de Campos

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Primary splenic angiosarcoma (PSA) is a rare neoplasm of vascular origin associated with aggressive behavior and poor prognosis. The clinical presentation is usually non-specific and is mostly characterized by a wasting disease with anemia and splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old woman with cardiovascular comorbidities with a 6-month history of weight loss, fatigue, weakness, pallor, and abdominal pain. The physical examination showed massive splenomegaly and pallor. After a thorough evaluation that ruled out lymphoproliferative diseases, the working diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the emergency room with severe abdominal pain, worsening fatigue, and a remarkable pallor. Point-of-care ultrasound showed free intraperitoneal fluid. Spleen rupture was confirmed by abdominal computed tomography (CT) scan, and an emergency laparotomy with splenectomy was performed. The postoperative period was uneventful, and the patient recovered in a few days. The histopathology confirmed the diagnosis of PSA and the patient was referred to an oncological center. Two months later staging CT demonstrated liver and peritoneal metastases, and despite the chemotherapy she died 6 months after the diagnosis.


Splenic Diseases, Splenomegaly, Hemangiosarcoma, Splenic Rupture


Xu L, Zhang Y, Zhao H, Chen Q, Ma W, Li L. Well-differentiated angiosarcoma of spleen: a teaching case mimicking hemangioma and cytogenetic analysis with array comparative genomic hybridization. World J Surg Oncol. 2015;13(1):300. 10.1186/s12957-015-0716-1.26462621

Hamid KS, Rodriguez JA, Lairmore TC. Primary splenic angiosarcoma. JSLS. 2010;14(3):431-5. 10.4293/108680810X12924466006521.21333203

Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology. 2005;235(1):106-15. 10.1148/radiol.2351040308.15749977

Li R, Li M, Zhang LF, et al. Clinical characteristics and prognostic factors of primary splenic angiosarcoma: a retrospective clinical analysis from China. Cell Physiol Biochem. 2018;49(5):1959-69. 10.1159/000493656.30235449

Azevedo OS, Santos BN, Liboni NS, Costa JF, Campos OD. Splenic angiosarcoma: a diagnostic splenectomy finding. Case Rep Oncol. 2016;9(3):733-7. 10.1159/000452619.27920710

Langhans T. Pulsirende cavernöse Geschwulst der Milz mit metastatischen Knoten in der Leber. Tödtlicher Verlauf binnen 5 Monaten. Arch Pathol Anat Physiol Klin Med. 1879;75(2):273-91. 10.1007/BF02134657.

Falk S, Krishnan J, Meis JM. Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. Am J Surg Pathol. 1993;17(10):959-70. 10.1097/00000478-199310000-00001.8372948

Shukla M, Shukla VK, Basu S, Kumar M. Fever, anemia and splenomegaly: a rare presentation of splenic angiosarcoma. Indian J Med Paediatr Oncol. 2011;32(4):230-2. 10.4103/0971-5851.95148.22563160

Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983-91. 10.1016/S1470-2045(10)70023-1.20537949

Kohutek F, Badik L, Bystricky B. Primary angiosarcoma of the spleen: rare diagnosis with atypical clinical course. Case Rep Oncol Med. 2016;2016:4905726. 10.1155/2016/4905726.27867672

Takato H, Iwamoto H, Ikezu M, Kato N, Ikarashi T, Kaneko H. Splenic hemangiosarcoma with sinus endothelial differentiation. Acta Pathol Jpn. 1993;43(11):702-8.8310831

Neuhauser TS, Derringer GA, Thompson LDR, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. Mod Pathol. 2000;13(9):978-87. 10.1038/modpathol.3880178.11007038

Yang KF, Li Y, Wang DL, Yang JW, Wu SY, Xiao WD. Primary splenic angiosarcoma with liver metastasis: a case report and literature review. World J Gastroenterol. 2016;22(12):3506-10. 10.3748/wjg.v22.i12.3506.27022233

Deng R, Chang W, Wu X, Chen J, Tao K, Zhang P. Primary splenic angiosarcoma with fever and anemia: a case report and literature review. Int J Clin Exp Pathol. 2015;8(11):14040-4.26823717

Coppola S, Leva A, Pagni F, Famularo S, Gianotti L. Demanding diagnosis of splenic angiosarcoma as cause of delayed treatment of spontaneous splenic rupture: a case report and literature review. Case Rep Surg. 2017;2017:6256102. 10.1155/2017/6256102.28261515

Serrano OK, Knapp E, Huang K, et al. Pediatric primary splenic angiosarcoma: an aggressive multidisciplinary approach to the oncologic management of a rare malignancy. World J Surg Oncol. 2014;12(1):379. 10.1186/1477-7819-12-379.25487642

Badiani R, Schaller G, Jain K, Swamy R, Gupta S. Angiosarcoma of the spleen presenting as spontaneous splenic rupture: a rare case report and review of the literature. Int J Surg Case Rep. 2013;4(9):765-7. 10.1016/j.ijscr.2013.06.007.23856255

Naka N, Ohsawa M, Tomita Y, et al. Prognostic factors in angiosarcoma: a multivariate analysis of 55 cases. J Surg Oncol. 1996;61(3):170-6. 10.1002/(SICI)1096-9098(199603)61:3<170::AID-JSO2>3.0.CO;2-8.8637202

Chen KT, Bolles JC, Gilbert EF. Angiosarcoma of the spleen: a report of two cases and review of the literature. Arch Pathol Lab Med. 1979;103(3):122-4.581838

Aqil B, Green LK, Lai S. Primary splenic angiosarcoma associated with anemia, leukocytosis and thrombocytopenia. Ann Clin Lab Sci. 2014;44(2):217-21.24795063

Abbott RM, Levy AD, Aguilera NS, Gorospe L, Thompson WM. From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics. 2004;24(4):1137-63. 10.1148/rg.244045006.15256634

Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology. 2005;235(1):106-15. 10.1148/radiol.2351040308.15749977

Valbuena JR, Levenback C, Mansfield P, Liu J. Angiosarcoma of the spleen clinically presenting as metastatic ovarian cancer. A case report and review of the literature. Ann Diagn Pathol. 2005;9(5):289-92. 10.1016/j.anndiagpath.2005.03.007.16198958

Hsu JT, Chen HM, Lin CY, et al. Primary angiosarcoma of the spleen. J Surg Oncol. 2005;92(4):312-6. 10.1002/jso.20419.16299797

Duan YF, Jiang Y, Wu CX, Zhu F. Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review. World J Surg Oncol. 2013;11(1):53. 10.1186/1477-7819-11-53.23497454

Singh P, Sharma S, Gupta P, Lal A, Srinivasan R. Primary splenic angiosarcoma with liver metastasis: a rare neoplasm diagnosed on fine-needle aspiration cytology and cell block immunocytochemistry. J Cytol. 2018;35(2):114-6. 10.4103/JOC.JOC_148_16.29643660

Casper ES, Waltzman RJ, Schwartz GK, et al. Phase II trial of paclitaxel in patients with soft-tissue sarcoma. Cancer Invest. 1998;16(7):442-6. 10.3109/07357909809011697.9774950

Zwi LJ, Evans DJ, Wechsler AL, Catovsky D. Splenic angiosarcoma following chemotherapy for follicular lymphoma. Hum Pathol. 1986;17(5):528-30. 10.1016/S0046-8177(86)80044-2.3516861

Hara T, Tsurumi H, Kasahara S, et al. Long-term survival of a patient with splenic angiosarcoma after resection, high-dose chemotherapy, and autologous peripheral blood stem cell transplantation. Intern Med. 2010;49(20):2253-7. 10.2169/internalmedicine.49.3969.20962445

Koontz BF, Miles EF, Rubio MA, et al. Preoperative radiotherapy and bevacizumab for angiosarcoma of the head and neck: two case studies. Head Neck. 2008;30(2):262-6. 10.1002/hed.20674.17685450

Miura H, Asada Y. Treatment of eyelid lesion of angiosarcoma with facial artery recombinant interleukin-2 (rIL-2) injection. J Am Acad Dermatol. 2006;54(5):907-8. 10.1016/j.jaad.2005.11.1047.16635681

Takeuchi K, Deguchi M, Hamana S, Motoyama S, Kitazawa S, Maruo T. A case of postirradiation vaginal angiosarcoma treated with recombinant interleukin-2 therapy. Int J Gynecol Cancer. 2005;15(6):1163-5. 10.1111/j.1525-1438.2005.00268.x.16343203

Skubitz KM, Haddad PA. Paclitaxel and pegylated-liposomal doxorubicin are both active in angiosarcoma. Cancer. 2005;104(2):361-6. 10.1002/cncr.21140.15948172

Amaya CN, Perkins M, Belmont A, et al. Non-selective beta blockers inhibit angiosarcoma cell viability and increase progression free- and overall-survival in patients diagnosed with metastatic angiosarcoma. Oncoscience. 2018;5(3-4):109-19.29854879




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