Rhabdomyosarcoma

Fig. 2.18. Sinonasal malignant epithelioid schwannoma: marked S-100 protein positivity in a large cell malignant neoplasm, mimicking amelanotic melanoma

ICD-O:8900/3

Rhabdomyosarcoma is the most common sinonasal malignancy of the paediatric age, but it is also observed in adults [37, 116]. The most common histologic subtypes are the embryonal and the alveolar [37]. The overall 5-year survival is around 40%; adult age and alveolar subtype are adverse prognostic factors. Treatment includes a combination of radiotherapy and chemotherapy, with surgical resection reserved for residual disease. The risk of neck involvement is high.

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