Infantile Extracranial Rhabdoid Tumor of the Scalp

Extracranial rhabdoid tumor is a rare tumor that can originate in multiple organs, and it is most commonly seen in the kidneys. This tumor has a grave prognosis. We report to the best of our knowledge the first case of infantile scalp extracranial rhabdoid tumor in a 6-month-old male baby who presented with a right parietal scalp mass since the age of 1 month. This mass was initially diagnosed as scalp hemangioma based on clinical and imaging findings. However, this mass was growing fast which necessitated excision. Pathologic examination after excision showed a malignant tumor composed of sheets of rhabdoid cells. Immunohistochemically, this tumor tested positive for vimentin, CD 99, glypican-3, synaptopysin, WT-1, CK, and EMA. INI-1 immunostain was lost in the tumor cells. Subsequently, this tumor was pathologically diagnosed as extracranial scalp rhabdoid tumor. After tumor excision, the patient was referred to pediatric oncology to receive chemotherapy. Experience with scalp extracranial rhabdoid tumors is limited. However, this tumor in other organs carries a grave prognosis. Although scalp extracranial rhabdoid tumor is an extremely rare tumor, it should be kept in mind in the differential diagnosis of infantile scalp masses given the need of combined surgical and chemotherapeutic treatment.