Esophageal Hemangiopericytoma or Hemangiopericytoma-Like Tumor: A Case Report and Review of the Literature

Acta Clinica Croatica; Vol.43 No.3 09/2004; 43(3).
Source: OAI


Hemangiopericytoma is a rare malignant vascular tumor deriving from pericytes. It usually occurs in the extremities and retroperitoneum, and extremely rarely in the gastrointestinal tract. A case of hemangiopericytoma in the gastroesophageal region is reported. A 56-year-old man was treated with H2-receptor antagonist for dyspeptic symptoms that had occurred three months before. Therapy failed to prove successful and gastroendoscopy was performed in March 2001 to reveal slightly reddish ventricular mucosa with normal rugal folds and a sessile, polypoid, grayish mass of rough surface located at the gastroesophageal junction. Histopathologic examination of polypoid mass samples revealed a vascular tumor covered with squamous epithelium. The hallmarks of the tumor were numerous branching, slit-like capillary channels with "staghorn" appearance, and large gaping sinusoidal spaces enclosed within nests of ovoid cells. Vascular invasion, mitotic activity, giant cells and necrosis were not found. Immunohistochemical analysis showed diffuse and strong reactivity for vimentin and focal, low or moderate reactivity for actin, S-100 and smooth muscle actin. Proliferation index measured by PCNA and Ki-67 ranged from 2% to 13% (median 11.2%) and from 5% to 18% (median 7.6%), respectively. A diagnosis of hemangiopericytoma with low malignant potential was made. Almost two years of the intervention, the patient is alive and free from any signs of tumor spread.

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