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Schwannoma 9560/0

Neurofibroma 9540/0

Malignant peripheral nerve sheath tumour (MPNST) 9540/3

Both benign (especially schwannoma and neurofibroma) and malignant peripheral nerve tumours (MPNST) can occur in the larynx, although vanishingly rare. Any age group can be affected and there is no gender predilection. Association of neurofibroma with neurofibromatosis-1 (NF1) has been reported in the larynx {385,2508}.

Macroscopically, benign and malignant PNST most often involve the supraglottis in relation to the superior laryngeal nerve. The tumours are of variable size and present as smooth, round or lobulated to fusiform submucosal masses, often demonstrating cystic or mucinous degeneration. The mucosa is usually intact, although larger tumours may ulcerate. Schwannoma is encapsulated and solitary with the nerve of origin attached peripherally. In contrast, neurofibroma is non-encapsulated, occasionally multiple or plexiform, especially when it is associated with NF1 and expands the nerve in a fusiform fashion rather than pushing it aside. MPNST are infiltrative, mitotically active and often ulcerate the mucosa The tumour cells are immunoreactive, often patchy, with S-100 protein, vimentin, epithelial membrane antigen (EMA) and Leu-7. Distinguishing benign from malignant tumours on small biopsies may be difficult {385,659,1202,1235,1974,2508, 2591}.

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