mart-1-antigen and Urinary-Bladder-Neoplasms

To study the clinicopathologic features and differential diagnosis of perivascular epithelioid cell tumors (PEComas) occurring in the urinary system.. The clinicopathologic features of 21 cases of PEComa from September 2002 to September 2010 occurring in the urinary system were retrospectively reviewed. Immunohistochemical study for HMB 45, S-100 protein, smooth muscle actin, desmin, Melan A and Ki-67 was carried out.. Amongst the 21 cases studied, there were 5 males and 16 females. The age of patients ranged from 16 to 76 years (median = 51.3 years). Twenty cases occurred in the kidney and 1 in the bladder. The predominant histopathologic subtype of renal PEComas was classic type (10/20), followed by epithelioid type (5/20), smooth muscle type (3/20), inflammatory type (1/20) and sclerosing type (1/20). Immunohistochemical study showed that HMB 45 and smooth muscle actin were positive in 95.2% (20/21) and 80.9% (17/21) cases, respectively. Melan A, desmin and S-100 protein were expressed in 71.4% (15/21), 61.9% (13/21) and 33.3% (7/21) cases, respectively. The mean proliferative index was 1.29% (range = 0 to 5%). HMB 45 and Melan A were expressed in all of the 5 cases of epithelioid PEComas, whereas smooth muscle actin and desmin were only expressed in one of them. There was no significant difference between epithelioid PEComas and non-epithelioid PEComas in the expression of HMB 45, Melan A, smooth muscle actin and desmin. Positive staining for HMB 45 and smooth muscle actin was demonstrated in the case of bladder PEComa.. PEComas of the urinary system predominantly affect the kidney. Epithelioid renal PEComas and bladder PEComa are relatively rare and have unique pathologic features. It is necessary to distinguish PEComas from other malignant tumors. Immunohistochemical study for HMB 45, Melan A and smooth muscle actin is helpful for confirmation of diagnosis.

Topics: Actins; Adolescent; Adult; Aged; Carcinoma, Renal Cell; Desmin; Diagnosis, Differential; Female; Gastrointestinal Stromal Tumors; gp100 Melanoma Antigen; Humans; Immunohistochemistry; Kidney Neoplasms; Leiomyosarcoma; Male; MART-1 Antigen; Melanoma; Melanoma-Specific Antigens; Middle Aged; Perivascular Epithelioid Cell Neoplasms; S100 Proteins; Urinary Bladder Neoplasms; Young Adult

Perivascular epithelioid cell tumors are a family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis, clear cell sugar tumor of the lung and a group of rare morphologically and immunophenotypically similar lesions that show immunoreactivity for both melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. This case is presented for its rare occurrence and characteristic morphologic and immunohistochemical features.

Topics: Adult; Biomarkers, Tumor; Biopsy; gp100 Melanoma Antigen; Hematuria; Humans; Immunohistochemistry; Male; MART-1 Antigen; Melanoma-Specific Antigens; Perivascular Epithelioid Cell Neoplasms; Urinary Bladder Neoplasms