Alveolar rhabdomyosarcoma

Dr Henry Knipe and A.Prof Frank Gaillard et al.

Alveolar rhabdomyosarcomas are a type of rhabdomyosarcoma and account for 20-40% of all rhabdomyosarcomas 1-2.

Unlike embryonal rhabdomyosarcomas, which are more common, these tumours occur in slightly older individuals, typically 10-25 years of age 1

Although these tumours can be found essentially anywhere, they are more frequently situated in the deep compartments of the extremities 1-2.

Specific genetic abnormalities have been identified, that are specific for alveolar rhabdomyosarcomas. They include t(2;13) and t(1;13) chromosomal translocations resulting in PAX3-FKHR and PAX7-FKHR gene fusions 2.

Compared to the other two types (embryonal and pleomorphic rhabdomyosarcomas) it has the worst prognosis 1.

The features are non-specific (see radiographic features in the parent article: rhabdomyosarcoma), although in general alveolar rhabdomyosarcomas tend to have multiple areas of necrosis 1.

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Article information

rID: 8325
Tags: cases, cases, refs
Synonyms or Alternate Spellings:
  • Alveolar rhabdomyosarcomas
  • Alveolar type rhabdomyosarcoma

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