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Case Diagnosis and Discussion


Epithelioid Angiosarcoma of the duodenum and stomach.


Angiosarcoma involving the gastrointestinal tract is very rare, is often multicentric, and may involve the gastrointestinal tract as a primary tumor, a metastatic tumor, or by direct extension from a retroperitoneal tumor [1]. Gastrointestinal tract angiosarcoma usually presents with gastrointestinal bleeding, anemia, and/or melena and due to this non-specific presentation, there may be a delay in the initial diagnosis [1]. These tumors more commonly involve the colon and small intestine and are rare in the stomach [1].  Gastrointestinal tract angiosarcomas have a dismal prognosis and most patients die within 6 months, usually of uncontrollable hemorrhage [1].

The epithelioid variant of angiosarcoma, originally described in cutaneous tumors by Rosai et al. [2], has a propensity to masquerade as other tumors, particularly primary or metastatic poorly differentiated carcinoma, as well as melanoma, lymphoma, or mesothelioma [1]. Confounding this diagnostic pitfall, the epithelioid variant of angiosarcoma is well-known to aberrantly express cytokeratins [1, 3]. CD31 is a reliable immunohistochemical stain to resolve this differential diagnosis and is consistently positive in epithelioid angiosarcoma [1].

Endoscopically, gastrointestinal tract angiosarcomas are described as red or red-brown, polypoid mucosal-based lesions [1]. Histologically, epithelioid angiosarcomas of the gastrointestinal tract are composed of diffuse sheets of epithelioid cells with subtle clefting suggestive of vascular differentiation that fill the lamina propria [1]. The glands are typically preserved and there may be overlying erosions [1]. Cytologically, the cells are epithelioid with amphophilic to eosinophilic cytoplasm and hyperchromatic nuclei with prominent nucleoli [1]. There may be occasional intracytoplasmic lumina containing red blood cells [1].

Although angiosarcoma is a very rare tumor of the gastrointestinal tract, it is important to be aware of this entity and its ability to impersonate a poorly differentiated carcinoma, both morphologically and immunophenotypically; this is especially true when dealing with small endoscopic biopsy material.

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1. Allison KH, Yoder BJ, Bronner MP, Goldblum JR, Rubin BP. Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol. 2004;28(3): 298-307.
2. Rosai J, Sumner HW, Kostianovsky M, Perez-Mesa C. Angiosarcoma of the skin. A clinicopathologic and fine structural study. Hum Pathol. 1976;7(1):83-109.
3. Fletcher CDM, Beham A, Bekir S, Clarke AMT, Marley NJE. Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol. 1991;15(10):915-924.

This case was presented in abstract format at the College of American Pathologists 2013 annual meeting in San Diego, CA.