Perivascular epithelioid cell tumors (PEComas) in the pediatric population are very rare and frequently arise in the gastrointestinal tract. These tumors are characterized by variable morphological and immunohistochemical features, which may pose significant problems in differential diagnosis with other gastrointestinal mesenchymal tumors in pediatric patients. In presenting an unusual pediatric case of gastrointestinal PEComa, we also discuss the possible differential diagnosis of this rare entity.
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