RESUMO
Congenital plexiform neurofibroma is regarded as pathognomonic of neurofibromatosis (NF) especially when it is large and involves a major nerve trunk with changes of elephantiasis neuromatosa. Only very rarely malignant changes have been reported in cases who have NF for less than 5 years. 'Borderline' lesions are seen especially in patients with NF. In such cases criteria of mitotic activity should be utilized in establishing the diagnosis of malignancy. A case of 3 month child with congenital plexiform neurofibroma involving neck with elephantiasis neuromatosa with sarcomatous nodule has been described in the present article. The criteria for malignancy in nerve sheath tumors have also been discussed.
Assuntos
Dorso , Humanos , Lactente , Masculino , Pescoço , Neoplasias Primárias Múltiplas/congênito , Neurofibroma Plexiforme/congênito , Neurofibrossarcoma/congênito , Neoplasias de Tecidos Moles/congênitoRESUMO
A male child aged six years presented with a swelling in neck and hypertension. He was diagnosed to have and operated for Carotid body paraganglioma with Coexistent pheochromocytoma. Hypertension persisted even after excision of both the tumours which could be controlled with anti-hypertensive agents. The case is discussed with review of literature.