DOI: http://dx.doi.org/10.18203/2349-2902.isj20202438

Primary splenic angiosarcoma: a diagnostic enigma

Sameer A. Rege, Yogesh Takalkar, Amiteshwar Singh, Archana N. Rijhsinghani

Abstract


Primary splenic angiosarcomas (PSA) arise from splenic endothelium are rare and impose a diagnostic challenge preoperative. They can present as asymptomatic splenomegaly however; the commonest presentation is abdominal pain. The spleen can rapidly increase in size and can manifest as spontaneous rupture which would cause peritoneal dissemination of disease. Early metastasis of PSA is seen in liver, lungs, lymph nodes and gastrointestinal system. Preoperative diagnosis requires a high index of suspicion and ultrasound, contrast enhanced computerized tomography may essential to differentiate from splenic hemangioma. Splenic angiosarcoma are best treated with splenectomy with a limited disease, with care taken not to rupture and cause spillage. PSA are resistant to adjuvant radiation and chemotherapy. Mortality is high with median survival rate of only 5 months, irrespective of treatment and hence the need to diagnose before complications. Bisphosphonates, adjuvant radiation with chemotherapy have been attempted to increase disease free survival. We report a case of PSA emphasizes on early preoperative diagnosis to avoid progression of the disease.


Keywords


Primary splenic angiosarcoma, Mesenchymal splenic tumor, Splenic angiosarcoma, Vascular tumor of spleen

Full Text:

PDF

References


Shon W, Jenkins SM, Ross DT. Angiosarcoma: a study of 98 cases with immunohistochemical evaluation of TLE3, a recently described marker of potential responsiveness. J Cutaneous Pathology. 2011;38(12):961-6.

Krishnan FSJ, Meis JM. Primary angiosarcoma of spleen. A clinicopathological study of 40 cases. Am J Surg Pathol. 1993;17:959-70.

Neuhauser TS, Derringer GA, Thompson LD. Splenic angiosacroma: a clinicopathological and immunophenotypic study of 28 cases. Mod Pathol. 2000;13(9):978-87.

Langhans T. Pulsating cavernous neoplasm of spleen with metastatic nodules to the liver. Virchows Arch Pathol Anal. 1879;75:273-91.

Coppola S, Leva A, Pagni F. Demanding diagnosis of splenic angiosarcoma as cause of delayed treatment of spontaneous splenic rupture: A case report and literature review. Case Reports Surg. 2017:5.

Abbott RM, Levy AD, Aguilera NS, Gorospe L, Thompson WM. Primary vascular neoplasms of spleen: radiologic-pathologic co-relation. Radiographics. 2004; 24:1137-63.

Thompson WM, Levy Ad, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of spleen: imaging characteristics in 12 patients. Radiology. 2005;235: 106-15.

Hsu JT, Ueng SH, Hwang TL, Chen HM, Jan YY, Chen MF. Primary angiosarcoma of spleen in a child with long term survival. Pediatr Surg Int. 2007;23:807-10.

Gupta A, Patnaik MM, Naina HV. Angiosarcoma of prostate gland following brachytherapy for prostatic adenocarcinoma. Curr Urol. 2014;8:109-12.

Hai SA, Genato R, Gressel I, Khan P. Primary splenic angiosarcoma: case report and literature review. J Natl Med Assoc. 2000;92:143-6.

Arber DA, Strickler JG, Chen YY, Weiss LM. Splenic vascular tumors: a histologic, immunophenotypic and virologic study. Am J Surg Pathol. 1997;21:827-35.

Ginley MK, Googe P, Hanna W, Bell J. Primary angiosarcoma of spleen: a case report and review of literature. South Med J. 1995;88:873-5.

Smith VC, Eisenberg BL, Donald MEC. Primary splenic angiosarcoma, Case report and literature review. Cancer. 1985;55:1625-7.

Maier A, Bataille F, Krenz D, Anthuber M. Angiosarcoma as a rare differential diagnosis in spontaneous rupture of spleen. Chirurg. 2004;75(1):70-4.

Christopher SN. Frontario, Anna Goldenberg-Sandau, Darshan Roy, Roy Sandau. Primary splenic angiosarcoma presenting as idiopathic thrombocytopenic purpura: a case report and review of literature. Case Reports in Surgery. 2016:6.

Ozturk E, Mutulu H, Sonmoez G, Sildiroglu HO. Primary splenic angiosarcoma of spleen. Turkish J Gastroenterology. 2007;18(4):272-5.

Metser U, Miller E, Kessler A, Lerman H. Solid Splenic Masses: Evaluation with 18F-FDG PET/CT. J Nuclear Med. 2005;46(1):52-9.

Naka N, Ohsawa M, Tomita Y. prognostic factors in angiosarcoma: a multivariate analysis of 55 cases. J Surg Oncol. 1996;61:170-6.

Hu S, Ramos BCE, Verstovsek S. Metastatic splenic angiosarcoma presenting with thrombocytopenia and bone marrow fibrosis mimicking idiopathic thrombocytopenic purpura and primary myelofibrosis: a diagnostic challenge. Clinical Lymphoma Myeloma and Leukemia. 2013;13(5):629-33.

Bilski M, Surdyka D, Pasnik I. Adjuvant radiochemotherapy with a 23 months overall survival time in a patient after surgery due to splenic hemangiosarcoma rupture: a case report with literature review. Case Reports Oncological Med. 2018:5.

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