Clinicopathological study of the alveolar soft part sarcoma with 16 cases reports / 临床与实验病理学杂志

ABSTRACT

Purpose To investigate the clinicopathological changes, immuniohistochemistry and molecular genetics phenotypic charac-teristics of the alveolar soft part sarcoma ( ASPS) . Methods 16 cases of ASPS were studied with clinicopathological, cytochemistry technique and immunohistochemical staining, two cases of ASPS were studied by FISH. Results There were 6 males and 10 females with the age 8~58 years (median age 31. 7 years). The tumors were located at limbs, shoulder and back, tongue, vocal cords, lung, cervix, and ureter. The clinical manifestations of the patients was a slowly growing mass. Histopathologically the tumor showed typical organ-like or acinar-like structure with sinus-like blood vessels and the fibrous septa formation. Sometime the clear or abundant eosino-philic granular cytoplasm of the tumors were obvious. The tumor cells had a crystalline substance formation by PAS staining. The tumor cells were positive for TFE3 and Cathepsin K by immunohistochemical staining. The ASPL-TFE3 gene fusion detection of tumor cells were present. Conclusion ASPS often located on the limbs of young patients. It may misdiagnosed as malignant epithelial tumors, primary or metastatic adenocarcinoma and paraganglioma when the tumor locate on a rare anatomical parts or an organs, such as tongue, vocal cords, cervix, ureter, etc. It is valueable that the typical alveolar-like structure of the tumor and the expression for TFE3 and Cathepsin K for the pathological diagnosis of ASPS. It is an important indicator that the ASPL-TFE3 gene fusion detection by FISH for the tumor.