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Rhabdomyosarcoma in Adult
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Rhabdomyosarcoma in Adult

Contributors: Vivian Wong MD, PhD, Whitney A. High MD, JD, MEng, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Cynthia Christy MD
Other Resources UpToDate PubMed


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.


C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

302847003 – Rhabdomyosarcoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Ewing sarcoma
  • Lymphoma
  • Neuroblastoma
  • Melanoma
  • Cutaneous leiomyosarcoma
  • Neuroblastoma
  • Merkel cell carcinoma
  • Fibrosarcoma
  • Undifferentiated pleomorphic sarcoma
  • Angiosarcoma of skin
  • Poorly differentiated carcinoma
For perianal rhabdomyosarcoma:
  • Genital wart
  • Perianal cyst
  • Perianal abscess

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Last Updated:01/23/2022
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Rhabdomyosarcoma in Adult
A medical illustration showing key findings of Rhabdomyosarcoma : Abdominal mass, Bone pain, Oral tumor, Regional lymphadenopathy, Soft tissue swelling, Testicular mass, Neck mass, Pelvic mass, Pallor
Clinical image of Rhabdomyosarcoma - imageId=5246671. Click to open in gallery.  caption: 'A violaceous mass with overlying telangiectasia on the lower lip.'
A violaceous mass with overlying telangiectasia on the lower lip.
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