Abdominopelvic perivascular epithelioid cell tumor with overt malignancy: a case report.
Perivascular epithelioid cell tumor (PEComa) is a group of rare tumors composed of epithelioid cells with characteristic perivascular distribution and co-expression of the melanogenic marker HMB-45 and muscular markers. There are no documented parameters referring to the biologic behavior of PEComa. We report an abdominopelvic PEComa with overt malignancy in a 16-year-old girl. Histologically, the tumor showed the typical morphophenotypic characteristics of PEComa. Though the cytologic appearance of the tumor cells was relatively bland, the extensive necrosis, presence of lymph node metastases, and surrounding tissue invasion were all indicative of malignancy. Relapse of the tumor with multiple lymphadenopathy shortly after debulking surgery for the primary lesion, and postoperative adjuvant chemotherapy, further denoted its aggressive behavior.
Chen
I-Yu
IY
Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
Yang
Sheau-Fang
SF
Chen
Fang-Ming
FM
Chai
Chee-Yin
CY
D000008:Abdominal Neoplasms / Q000473:pathology*; D000293:Adolescent; D000951:Antigens, Neoplasm; D002277:Carcinoma / Q000473:pathology*; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D058950:Melanoma-Specific Antigens; D009361:Neoplasm Invasiveness; D009363:Neoplasm Proteins / Q000032:analysis; D010386:Pelvic Neoplasms / Q000473:pathology*
D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins