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C Le Fèvre, J Castelli, C Perrin, P L Hénaux, G Noël
Malignant peripheral nerve sheath tumours are extremely rare and can be associated with neurofibramatosis type 1. Their prognosis is poor and surgery remains the mainstay of therapy and should be the first line of treatment. Radiotherapy and chemotherapy are second line treatment and their effectiveness remains to demonstrate. The diagnosis is clinical, radiological, histological and immunohistochemical. Malignant peripheral nerve sheath tumours have a potential of local tumour recurrence very high and can metastasize...
April 2016: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
Elias Dominguez-Comesaña, Catherine Tome-Espiñeiro, Jose L Ulla-Rocha, Isabel Lorenzo-Lorenzo, Angel Lede-Fernandez, Jose L Portela-Serra
Carcinoids of the ampulla of Vater are infrequent tumors of which a quarter of cases have been detected in patients with type I neurofibromatosis. This hereditary disease is also associated with gastrointestinal stromal tumors (GIST). However, the coincidence of these three entities together have only been formerly detected in five cases. A 53 year-old female patient, diagnosed with type I neurofibromatosis, with a malignant carcinoid of ampulla of Vater and multiple gastrointestinal stromal tumors in the duodenum and jejunum, was treated with total pancreatectomy and the excision of her intestinal tumors...
September 2011: Asia-Pacific Journal of Clinical Oncology
A P Harper, H Y Yune, E A Franken
The renal diseases which cause systemic hypertension in the first two decades of life differ from the adult in their incidence and etiology. Seventeen patients (11 days to 20 years old), studied angiographically, demonstrated a wide spectrum of renal pathology including arterial thrombosis, fibromuscular hyperplasia, vasculitis, neurofibramatosis, cystic disease, pyelonephritis, Page kidney, and congenital anomalies.
April 1977: Radiology
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