Case ReportsObstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's diseaseSamonakis, D.N.a; Quaglia, A.b; Joshi, N.M.c; Tibballs, J.M.d; Nagree, A.c; Triantos, C.K.a; Davies, N.d; Standish, R.b; Dhillon, A.P.b; Davidson, B.R.a; Burroughs, A.K.a; Caplin, M.E.cAuthor Information aLiver Transplantation and Hepatobiliary Unit bHistopathology Department cNeuroendocrine Tumour Unit dRadiology Department, Royal Free Hospital, London, UK Correspondence and requests for reprints to Dr M.E. Caplin, BSc Hons, DM, FRCP, Neuroendocrine Tumour Unit, Centre for Gastroenterology, Royal Free Hospital, Pond Street, London NW3 2QG, UK Tel: +44 20 7830 2867; fax: +44 20 7472 6728; e-mail: M.Caplin@medsch.ucl.ac.uk Received 24 March 2005 Accepted 19 July 2005 European Journal of Gastroenterology & Hepatology: November 2005 - Volume 17 - Issue 11 - p 1229-1232 Buy SDC 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. © 2005 Lippincott Williams & Wilkins, Inc.