Rhabdomyosarcoma is a malignant mesenchymal tumor of skeletal muscle derivation. It is the most common soft tissue sarcoma in childhood and adolescence, but it is rare in adults. Rhabdomyosarcoma occurs most often upon the head / neck, on the extremities, in the gastrointestinal and genitourinary systems, and in the retroperitoneal and parameningeal areas.

Primary cutaneous rhabdomyosarcoma is exceedingly rare and is usually due to dermal invasion from deeper structures or a frank metastatic event. Secondary cutaneous rhabdomyosarcoma represents advanced disease with a poor prognosis.

Primary cutaneous rhabdomyosarcoma presents as a nonspecific papule or nodule. Several genetic syndromes and maternal factors are associated with an increased incidence of childhood rhabdomyosarcoma, including parental cocaine and marijuana use, Li-Fraumeni syndrome, neurofibromatosis type 1 (NF1), Beckwith-Wiedemann syndrome, and Costello syndrome.

Rhabdomyosarcoma has a slight predilection for male and White individuals. It has a generally poor prognosis due to a propensity for early lymphatic and hematologic metastases. Early diagnosis affords improved survival.

Controversy exists regarding classification of rhabdomyosarcoma, but the alveolar variant represents the most common cutaneous form, and it has an aggressive nature and adverse prognosis.