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Інтраневральна периневріома

ORPHA:100003· ICD-10 D36.1· Intraneural perineurioma

Визначення(English summary)

Intraneural perineurioma is a rare tumor of cranial and spinal nerves arising from peripheral nerve sheath and composed exclusively or predominantly of cells showing perineurial differentiation. It presents as a localized, tubular or fusiform enlargement of a nerve or nerve segment, usually in the extremities or the trunk, associated with a motor-predominant mononeuropathy including slow, painless, gradual loss of motor function in the involved nerve trunk with muscle weakness and atrophy and, rarely, sensory dysfunction. Cranial nerve involvement is rare.

Поширеність
<1 / 1 000 000
Вік початку
Adolescent, Adult, Childhood