Abstract
Rhabdomyosarcoma (RMS) is a rare soft tissue tumor with the most common sites of origin in the genitourinary tract, head, and neck regions, and extremities are less commonly involved. Spindle cell RMS is a rare variant of RMS in infants. RMS is uncommon in the hand. We present a case of spindle cell RMS in the hand of a full-term female newborn with regional lymph node involvement. Based on histopathology, the diagnosis of spindle cell RMS was made in stage IRS 3 of the Intergroup Rhabdomyosarcoma Study Group (IRSG) staging system. The infant underwent complete surgical resection and axillary lymph node removal, followed by chemotherapy. She died from complications of chemotherapy a year after initiation of treatment. The age, gender, poor outcome, and site are unusual features for this type of RMS.