Malignant Schwannoma with Rhabdomyoblastic Differentiation Malignant Schwannoma with Rhabdomyoblastic Differentiation a very rare variant of the malignant tumors arising in the peripheral nerve sheath. In 70% of the cases, the neoplasm is associated with Von Recklinghausen's neurofibromatosis; in the remaining 30% it is the sole morbid finding. Its biological behavior is, in any case, more aggressive than that of simple malignant schwannoma; its course is rapid and its prognosis is dismal. Local recurrences are frequent, and distant metastases, preferentially situated in the lung and brain, are present in about 50% of the cases. Post-operative radiotherapy is always indicated. [ Back ]

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