title: Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease.
abstract: 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.
authors.affiliation: Liver Transplantation and Hepatobiliary Unit, Royal Free Hospital, London, UK.
authors.forename: D N
authors.initials: DN
authors.lastname: Samonakis
authors.forename: A
authors.initials: A
authors.lastname: Quaglia
authors.forename: N M
authors.initials: NM
authors.lastname: Joshi
authors.forename: J M
authors.initials: JM
authors.lastname: Tibballs
authors.forename: A
authors.initials: A
authors.lastname: Nagree
authors.forename: C K
authors.initials: CK
authors.lastname: Triantos
authors.forename: N
authors.initials: N
authors.lastname: Davies
authors.forename: R
authors.initials: R
authors.lastname: Standish
authors.forename: A P
authors.initials: AP
authors.lastname: Dhillon
authors.forename: B R
authors.initials: BR
authors.lastname: Davidson
authors.forename: A K
authors.initials: AK
authors.lastname: Burroughs
authors.forename: M E
authors.initials: ME
authors.lastname: Caplin
mesh_terms: D000328:Adult; D014670:Ampulla of Vater; D018278:Carcinoma, Neuroendocrine / Q000150:complications*; D003138:Common Bile Duct Neoplasms / Q000150:complications*; D006801:Humans; D041781:Jaundice, Obstructive / Q000209:etiology*; D008113:Liver Neoplasms / Q000150:complications; D008297:Male; D009378:Neoplasms, Multiple Primary; D018318:Neurofibroma, Plexiform / Q000150:complications; D009456:Neurofibromatosis 1 / Q000150:complications*