Malignant extrarenal rhabdoid tumour in the oral cavity: Report of a rare malignancy in an unusual location

Malignant rhabdoid tumour (MRT) is a well established aggressive malignant neoplasm occurring in kidney particularly in infants and children. When this type of tumor involves any extrarenal site it is termed malignant extrarenal rhabdoid tumor (MERT). Here we report a case of MERT involving the oral cavity. To the best of our knowledge, apart from us only one oral MERT has been documented. Due to their scarcity, prompt and bold diagnosis is quite challenging for a histopathologist. Confirmed final diagnosis must be given after thorough immunohistochemical workup. Confusions and obscurities regarding a definitive treatment policy further complicate management also. We adopted a multimodality approach comprising of surgery, radiotherapy and chemotherapy for our patient. She is disease free for last 30 months on regular followup and maintaining her normal daily activities.