51/75. Congenital plexiform neurofibroma with a sarcomatous nodule in a three month old child. 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. ( info) |
52/75. Plexiform schwannoma. We report the clinical, histopathologic, and immunohistopathologic features of two cases of plexiform schwannoma. This rare benign tumor is worthy of recognition because it can be misdiagnosed as plexiform neurofibroma. Differentiation from plexiform neurofibroma is important because the latter is pathognomonic of von Recklinghausen disease and carries a significant risk of malignant transformation. Plexiform schwannoma is, in contrast, not necessarily associated with von Recklinghausen disease and malignant transformation has never been observed. ( info) |
53/75. Plexiform neurofibroma: an unusual cause of neck lump in a child. Although relatively rare, neurofibromas have been described in many different sites. We present the unusual case of an 8-year-old girl with a plexiform neurofibroma situated deep to the sternomastoid muscle, and review the current literature with particular regard to the pathology and site of neurofibromas. ( info) |
54/75. CT findings of plexiform neurofibromatosis involving the ileum and its mesentery. We present a case of plexiform neurofibromatosis involving the ileum and its mesentery. Computed tomography (CT) scans showed a cluster of small soft-tissue density nodules, which represented a cross-sectional image of the enlarged peripheral nerves and wall thickening of the distal ileum. Trapped fat tissue was demonstrated between these enlarged nerves. Histopathologic studies of the resected specimen correlated well with CT findings. ( info) |
55/75. foot drop in a long-distance runner. An unusual presentation of neurofibromatosis. An athletic patient presented with a nontraumatic peroneal neuropathy that failed to resolve after a period of rest. A magnetic resonance image (MRI) showed a multilobulated mass in the course of the common peroneal nerve consistent with a plexiform neurofibroma. Surgical exploration revealed a mass, which coursed from the midthigh to the fibular neck, that was intimately involved with the fibers of the nerve bundle and had cystic degeneration with vesicles along its length. The authors recommend MRI as highly accurate in diagnosing unusual causes of peroneal neuropathy. ( info) |
56/75. Case report 828: Plexiform neurofibroma of the tibial nerve invading the medial and lateral gastrocnemius muscles and plantaris muscle. A 5-year-old boy presented with a several-year history of swelling of his calf and muscular weakness. MRI demonstrated abnormal signal and diffuse enlargement of the gastrocnemius muscle. Pathological examination revealed plexiform neurofibroma, and the diagnosis of neurofibromatosis was subsequently made. The manifestations of neurofibromatosis and the peripheral nerve sheath tumors encountered in this disease are discussed. Special attention is paid to the MR features of the plexiform neurofibroma which is characteristic of this disorder. ( info) |
57/75. neurofibroma: an unusual presentation. We report neurofibroma discovered incidentally in a woman who suffered trauma to her cheek. The lesion was manifest on CT as a haematoma within a parotid tumour. ( info) |
58/75. Laryngeal involvement in neurofibromatosis. Neurofibromas of the larynx are an uncommon component of neurofibromatosis, but should be considered in the differential diagnosis of patients with a submucosal supraglottic mass. Complete surgical excision is the treatment of choice; however, incomplete excision may be preferable to aggressive debilitating surgery. Plexiform neurofibroma differs from non-plexiform neurofibroma in that it is poorly circumscribed and highly infiltrative. tracheostomy may be necessary. Sarcomatous degeneration is reported and carries a poor prognosis. ( info) |
59/75. Laryngeal involvement in pediatric neurofibromatosis: a case report and review of the literature. A case of neurofibroma of the larynx occurring in generalized neurofibromatosis (von Recklinghausen's disease) is presented, and the previously reported pediatric cases are reviewed. Laryngeal involvement in neurofibromatosis is rare and the predominant signs and symptoms include dyspnea, stridor, loss or change of voice and dysphagia. Problems posed related to diagnosis, management and course of this infrequent laryngeal localization are discussed. ( info) |
60/75. Diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract involving a large segment of small intestine. A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb. ( info) |