Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease
Samonakis, D. N., Quaglia, A., Joshi, N. M., Tibballs, J. M., Nagree, A., Triantos, C. K., Davies, N., Standish, R. A., Dhillon, A. P., Davidson, B. R., Burroughs, A. K. and Caplin, M. E. 2005, Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease, European journal of gastroenterology and hepatology, vol. 17, no. 11, pp. 1229-1232.
Title
Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease
European journal of gastroenterology and hepatology
Volume number
17
Issue number
11
Start page
1229
End page
1232
Total pages
4
Publisher
Lippincott Williams & Wilkins Inc
Place of publication
Philadelphia, Pa.
Publication date
2005-11
ISSN
0954-691X
Summary
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.
Language
eng
Field of Research
110307 Gastroenterology and Hepatology 111203 Cancer Genetics