ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
Rhabdomyosarcoma - Skin in Infant/Neonate
Print
Other Resources UpToDate PubMed

Rhabdomyosarcoma - Skin in Infant/Neonate

Print Images (5)
Contributors: Vivian Wong MD, PhD, Whitney A. High MD, JD, MEng, Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Nancy Esterly MD
Other Resources UpToDate PubMed

Synopsis

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.

Codes

ICD10CM:
C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

SNOMEDCT:
302847003 – Rhabdomyosarcoma

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

For perianal rhabdomyosarcoma:

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 07/17/2015
Copyright © 2019 VisualDx®. All rights reserved.
Rhabdomyosarcoma - Skin in Infant/Neonate
Print 5 Images
View all Images (5)
(with subscription)
Rhabdomyosarcoma : Abdominal mass, Bone pain, Oral tumor, Regional lymphadenopathy, Soft tissue swelling, Testicular mass, Neck mass, Pelvic mass, Pallor
Clinical image of Rhabdomyosarcoma
Copyright © 2019 VisualDx®. All rights reserved.