A 60-year-old African-American woman presented with a history of several weeks of
increasing shortness of breath and weakness. On examination, she was hemodynamically
stable with heme-positive brown stool. Initial laboratory studies revealed a hemoglobin
concentration of 4 g/dL. Upper endoscopy showed active oozing from a 2-cm ulcerated
mass on the gastric incisura angularis ([Fig. 1]) and a 1-cm polypoid lesion in the second portion of the duodenum just proximal
to the major papilla ([Fig. 2]). Biopsies demonstrated markedly atypical endothelial proliferation suggestive of
a high-grade angiosarcoma versus exuberant granulation tissue adjacent to the ulcer
([Fig. 3]). Immunohistochemical stains were positive for CD31 ([Fig. 4]), vimentin, and pancytokeratin, consistent with epithelioid angiosarcoma (EAS).
A positron emission tomography scan showed hypermetabolic foci in the stomach and
in the region of the duodenum that were highly suggestive of malignancy; no other
areas were identified. Due to continued gastrointestinal bleeding and the above findings,
the patient underwent an exploratory laparotomy with distal gastrectomy, partial duodenectomy,
and gastrojejunostomy. The final surgical pathological study confirmed the diagnosis
of EAS.
Fig. 1 Esophagogastroduodenoscopy demonstrating an ulcerated hemorrhagic polypoid lesion
on the incisura angularis of the stomach.
Fig. 2 Esophagogastroduodenoscopy revealing a polypoid lesion in the second portion of the
duodenum.
Fig. 3 Duodenum. Photomicrograph showing irregular, anastomosing vascular channels lined
by enlarged endothelial cells with epithelioid morphology (hematoxylin & eosin; original
magnification × 40).
Fig. 4 The neoplastic cells show positive immunoreactivity for CD31 (immunohistochemistry;
original magnification × 100).
Primary angiosarcoma of the gastrointestinal tract is extremely rare. These tumors
are characterized by an extremely aggressive course, with a high tendency to recur
and metastasize, leaving patients with a very poor prognosis [1]
[2]
[3]
[4]. Since treatment options for advanced disease are limited, complete surgical resection
remains the most crucial factor to achieving a favorable outcome, and this underscores
the importance of early and appropriate diagnosis. In patients who present with gastrointestinal
bleeding and in whom biopsy reveals a poorly differentiated malignancy, the diagnosis
of angiosarcoma should be considered.
Immunohistochemical stains and pathological expertise with gastrointestinal malignancies
are often necessary to differentiate angiosarcoma from other malignancies [2]
[3]. For patients with advanced disease, chemotherapeutic and antiangiogenesis agents
are being actively investigated and may have a palliative role, particularly for patients
who become transfusion-dependent [3]
[4].
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