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← Childhood Rhabdomyosarcoma

HEALTH PROFESSIONAL · SOURCE READING

Unusual primary sites

Source: Childhood Rhabdomyosarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 11, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.

Context: Treatment of Childhood Rhabdomyosarcoma / Surgery and RT by Primary Site of Disease (Local Control Management)

Rhabdomyosarcoma occasionally arises in sites other than those previously discussed.

Brain. Patients with localized primary rhabdomyosarcoma of the brain can occasionally be cured using a combination of tumor excision, RT, and chemotherapy.[162][Level of evidence C2]

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Larynx. Patients with laryngeal rhabdomyosarcoma will usually be treated with chemotherapy and RT after biopsy in an attempt to preserve the larynx.[163]

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Diaphragm. Patients with diaphragmatic tumors often have locally advanced disease that is not grossly resectable initially because of fixation to adjacent vital structures such as the lung, great vessels, pericardium, and/or liver. In such circumstances, chemotherapy and RT should be initiated after diagnostic biopsy. Removal of residual tumor at a later date if clinically indicated could be considered.[164]

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Ovary. Two well-documented cases of primary ovarian rhabdomyosarcoma (one Stage III and one Stage IV) have been reported to supplement the eight previously reported patients. These two patients were alive at 20 and 8 months after diagnosis. Six of the previously reported eight patients had died of their disease.[165][Level of evidence C2] Treatment with combination chemotherapy, followed by removal of the residual mass or masses, can sometimes be successful.[165]

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Preserved source evidence · Not medical advice