Malignant rhabdoid tumor arising in a mixed epithelial, stromal tumor of kidney: report of a male case, review of the literature

Abstract

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare biphasic tumor composed of both stromal and epithelial components, the latter showing a variable proportion of solid to cystic areas. These tumors show a marked female predominance, commonly occur in perimenopausal age groups, and often have an ovarian-type stroma with ER and PR positivity, suggesting steroids may play a role in pathogenesis. Although typically benign, rare cases showing malignant transformation have been reported. We present a case of a 42-year-old man with a 10 cm right kidney mass located in the renal pelvis. Histologically, the majority of the tumor was composed of a diffuse, sheet-like growth of malignant cells demonstrating a rhabdoid morphology with large nuclei, prominent nucleoli, and eosinophilic eccentric cytoplasm. Brisk mitotic activity and coagulative type necrosis was also noted. Intimately associated with this malignant rhabdoid component was a much smaller portion of tumor featuring variably sized bland epithelial tubules embedded within a stroma composed of bland spindle cells and areas of hyalinization, diagnostic of MEST. By immunohistochemistry, the malignant rhabdoid tumor portion of the neoplasm showed complete loss of nuclear INI-1, while the MEST component retained nuclear expression of this antigen. With these features taken together, our case represents a malignant rhabdoid tumor arising in a background of MEST. To our knowledge, this case represents the first case of a MEST showing malignant transformation in the form of malignant rhabdoid tumor in a male patient in the English language literature.

Keywords: Mixed epithelial and stromal tumor; and malignant rhabdoid tumor; kidney.