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First report of concomitant pheochromocytoma and duodenal neuroendocrine tumour in a sporadic multiple endocrine neoplasia type 1
  1. Banafsheh Motazedi1,
  2. Maziar Rahmani1,2,
  3. James M Welch3,
  4. Abbas Motazedi1
  1. 1Internal Medicine, Providence Health System, Washington, District of Colombia, USA
  2. 2Inter-Institute Endocrinology Fellowship Program, Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD), National Institute of Health (NIH), Bethesda, Maryland, USA
  3. 3Metabolic Diseases Branch, National Institute of Health (NIH), Bethesda, Maryland, USA
  1. Correspondence to Dr Abbas Motazedi, afmotazedi{at}


A 77-year-old woman was diagnosed with pheochromocytoma followed by adrenalectomy at age 57. Hyperparathyroidism without osteoprosis was diagnosed at age 58. At age 75, Dual Energy X-ray Absoptiometry (DEXA) revealed osteoporosis and sestamibi scan showed a left parathyroid adenoma. Criteria for parathyroidectomy were met, and she underwent parathyroidectomy. Furthermore, she presented with haematochezia at age 75. An abdominal CT demonstrated a mass in the second portion of the duodenum. Additionally, octreoscan revealed somatostatin receptor positive tissue in the duodenum and Gallium 68 dotatate scan also showed a well-differentiated duodenal neuroendocrine tumour (NET). Genetic testing for MEN1, MEN2 and MEN4 was negative. Diagnosis of sporadic MEN1 syndrome was made. The patient underwent resection of the duodenal NET at age 76. She is in good health 21 years after her first presentation of MEN1. In summary, we present the first sporadic case of MEN1 with concomitant pheochromocytoma and duodenal NET which occurred 20 years apart.

  • adrenal disorders
  • calcium and bone
  • neurogastroenterology
  • neuroendocrinology
  • endocrine cancer

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  • Contributors BM has assisted with acquisition of patient’s medical history and relevant medical data including but not limited to imaging studies/reports, surgical and pathological reports, reports of genetic studies, laboratory tests, etc. Additionally, she has drafted and critically revised the manuscript. MR and JMW have assisted in drafting, reviewing and analytical revision of the manuscript. AM has provided the final approval for the manuscript for publication. He will be the corresponding author for this manuscript. He is also agreeable to be accountable for all aspects of the work produced by the coauthors listed below and ensuring that all questions related to accuracy or integrity of any part of this work are appropriately investigated and resolved. Furthermore, he declares the below-named authors to be the only two authors who fulfil the criteria for authorship.

  • 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.

  • Patient consent Obtained.

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