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 was treated for recurrent peptic ulcer disease and later developed diabetes mellitus and chronic diarrhoea. A metastasising somatostatinoma was histologically proven and evidence of a concomitant gastrin-producing neuroendocrine tumour was found. Neuroendocrine tumours (NETs) are very rare neoplasms originating from a wide variety of endocrine and nervous system 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 of our knowledge. We discuss oncogenic pathomechanisms related to the underlying conditions and propose stringent monitoring for tumours in HIV-positive patients with phakomatoses as well as initiation of antiretroviral therapy.

Neurofibromatosis and HIV infection in a pregnant woman

“…de Castro et al described a 38‐year‐old woman with NF1 and AIDS with peripheral T‐cell lymphoma with good response to chemotherapy and Isa et al a 30‐year‐old woman with HIV infection whose disease remained stable with an adequate response to antiretroviral therapy. More recently, Hiesgen and Variava 5 discussed a 30‐year‐old woman with NF1 and somatostatinoma who died soon after diagnosis. Also, Forte 6 described a case of a 41‐year‐old female patient with NF1 and uncontrolled HIV infection with simultaneously supratentorial and infratentorial pilocytic astrocytoma.…”