Neuroendocrine tumour in a patient with neurofibromatosis type 1 and HIV

Abstract

We report the case of an HIV-positive female patient with neurofibromatosis type 1 who wastreated for recurrent peptic ulcer disease and later developed diabetes mellitus and chronicdiarrhoea. A metastasising somatostatinoma was histologically proven and evidence of aconcomitant gastrin-producing neuroendocrine tumour was found. Neuroendocrine tumours (NETs) are very rare neoplasms originating from a wide variety of endocrine and nervoussystem tissue with the ability to produce different hormones. A somatostatin- and gastrinsecreting NET in a patient with HIV has not been reported in the literature, to the best ofour knowledge. We discuss oncogenic pathomechanisms related to the underlying conditionsand propose stringent monitoring for tumours in HIV-positive patients with phakomatoses aswell as initiation of antiretroviral therapy.