Clinicopathologic analysis of 4 perivascular epithelioid cell tumors (PEComas) of the gastrointestinal tract.

Department of Pathology, The Chinese PLA General Hospital, Beijing, China.
International Journal of Surgical Pathology (Impact Factor: 0.96). 02/2009; 18(4):243-7. DOI: 10.1177/1066896908330481
Source: PubMed

ABSTRACT This study describes the clinical, histologic, and immunohistochemical features of four cases of GI PEComa. The size of the tumors ranged from 3.5 to 6.0cm in diameter, and all were located in wall of the large bowel. Microscopically, the tumors were characterized by an epithelioid arrangement of tumor cells, which had abundant clear to pale eosinophilic granular cytoplasm, vesicular nuclei and prominent nucleoli. The stroma was rich in capillaries, a sinusoidal vasculature and thick-walled blood vessels. Mitotic figures were generally rare. Immunohistochemically, the tumors were positive for Vimentin (4/4), HMB-45 (4/4), Melan-A(4/4),alpha-smooth muscle actin (4/4), and desmin (3/4). The patients have been well with no evidence of disease at 8, 15, 32 and 36 months,respectively, after the surgical operation. GI tract PEComas should be regarded as tumors of an uncertain malignant potential until long-term outcome data for a larger number of patients become available.

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