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Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature
  1. Subhanudh Thavaraputta1,
  2. Suzanne Graham2,
  3. Ana M Rivas Mejia3 and
  4. Joaquin Lado-Abeal3
  1. 1 Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas, USA
  2. 2 Department of Pathology, Texas Tech Univeristy Health Sciences Center, Lubbock, Texas, USA
  3. 3 Division of Endocrinology, Department of Internal Medicine, Texas Tech University Health Science Center School of Medicine, Lubbock, Texas, USA
  1. Correspondence to Dr Ana M Rivas Mejia, marcella.rivas{at}


Somatostatinomas are rare neuroendocrine tumours, mostly located in the pancreas or duodenum, with an estimated incidence of 1 in 40 million. Duodenal somatostatinomas (DSs) are usually found in association with neurofibromatosis type 1 (NF1), tuberous sclerosis and Von Hippel-Lindau syndrome. Gastrointestinal stromal tumours (GIST) have also been described in NF1, but the association with somatostatinoma is very uncommon. We report the case of a patient with NF1 who presented with obstructive jaundice due to multiple firm nodules around the ampulla of Vater. A pancreaticoduodenectomy was performed and revealed a 1 cm duodenal/ampullary mass which stained positive for somatostatin, together with a GIST also found on the duodenal wall. Despite its rarity, ampullary somatostatinomas should be considered in the differential diagnosis of biliary tract dilation in patients with NF1.

  • endocrine cancer
  • neuro genetics
  • neuroendocrinology

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  • Patient consent for publication Obtained.

  • Contributors ST: data acquisition, drafting manuscript, manuscript revision, gathering information, and literature review/search. SG: involved in a patient care, manuscript revision, image selection and interpretation. JL-A: involved in patient care, manuscript revision and final approval. ARM: manuscript revision, data acquisition and corresponding author.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.