신생아에서 연부 조직 종괴로 진단된 선천성 전신성 파종성 악성 횡문근종양 1례

Abstract

Malignant rhabdoid tumor (MRT) was first described as a “rhabdomyosarcomatoid” variant of Wilms tumor, but was later identified as a distinct entity. MRTs are extremely rare and highly aggressive neoplasm with poor outcome, occurring mostly in kidneys and central nervous system, less frequently in extrarenal sites. MRTs are referred to as “congenital” when it is diagnosed at or immediately after birth. There are few reports about congenital disseminated MRT at birth. We report a case of congenital malignant rhabdoid tumor presenting as a soft tissue mass in the right arm at birth although the autopsy revealed the systemic disseminated tumors through the whole body with typical microscopic findings