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Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease
journal contributionposted on 2005-11-01, 00:00 authored by D Samonakis, A Quaglia, N Joshi, J Tibballs, A Nagree, C Triantos, N Davies, Richard StandishRichard Standish, A Dhillon, B Davidson, A Burroughs, M Caplin
Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, with variable clinical manifestations and unpredictable course, associated with an increased incidence of various tumours. Plexiform neurofibromas are hallmark lesions of NF1; they are slow-growing tumours, which account for substantial morbidity, including disfigurement and functional impairment, and may even be life-threatening. Neuroendocrine tumours (NETs), a rare diverse group of neoplasms, are occasionally associated with neurofibromatosis. Pancreatic NETs are tumours with an incidence of less than 1/100 000 population/year and complex patterns of behaviour, which often need complicated strategies for optimal management. We present the case of a young adult with NF1, having a unique concurrence of plexiform neurofibroma involving the liver with an ampullary NET, and we discuss step by step the management in a specialist centre.