Malignant mixed Mullerian tumor with rhabdoid features: A report of two cases and a review of the literature

Rhabdoid tumors were originally described as a type of pediatric renal neop lasm that contains cells resembling rhabdomyoblasts but lacking muscle diff erentiation. Extrarenal rhabdoid tumors have since been reported in multipl e anatomic sites in the pediatric and adult population. These tumors are ch aracterized by an aggressive clinical course, resistance to treatment, and a rapidly fatal outcome. Eight cases of uterine neoplasms with rhabdoid dif ferentiation have been previously reported. In the three cases where clinic al follow-up was available, the patients died of disease within 3 to 17 mon ths after the diagnosis was established. We report two cases of uterine mal ignant mixed Mullerian tumor (carcinosarcoma) with rhabdoid differentiation . The findings and clinical outcome confirm the aggressive nature of uterin e tumors with rhabdoid differentiation. One of the patients died of disease 3 months after initial operative treatment while the other patient's tumor recurred in 1 month and she died within 10 weeks. The poor prognosis of th ese neoplasms makes their histopathologic recognition important. (C) 1999 A cademic Press.