Congenital malignant rhabdoid tumor of the scalp.

Background: Malignant rhabdoid tumors (MRT) are rare but aggressive tumors presenting in the pediatric population. First thought a variant of Wilms' tumor in the kidney, it is recognized as presenting at renal, central nervous system and other extra-renal primary sites. It is uniformly of very poor prognosis, however.

Methods: We present a case of congenital MRT of the scalp, which we believe to be the first described at this site. The clinical and histopathological features of the tumor are discussed in light of the current literature on MRT at other sites. The bleak prognosis at this site appears to be no different from others - the child succumbed at 10 months old despite surgical resection and initial excellent response to chemotherapy.

Conclusions: Malignant rhabdoid tumor has a very poor prognosis and needs to be considered in the differential diagnosis of similar lesions by clinicians involved in pediatric head and neck care.