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Rare and aggressive metastatic pheochromocytoma recurrence in a patient with MEN 2A syndrome
  1. Edrome Ferrer Hernandez and
  2. Nemencio Almare Nicodemus Jr
  1. Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Metro Manila, Philippines
  1. Correspondence to Dr Edrome Ferrer Hernandez; hernandezedrome{at}gmail.com

Abstract

An adult male in his early 30s diagnosed with multiple endocrine neoplasia type 2A syndrome, confirmed through genetic testing, presented as bilateral pheochromocytoma in a metachronous fashion, primary hyperparathyroidism and medullary thyroid carcinoma. Left and right adrenalectomy was done 9 years and 3 years ago, respectively. He was also subjected to total thyroidectomy with neck dissection and left inferior parathyroidectomy. During surveillance monitoring, 24-hour total urine metanephrines were elevated 13.977 mg (Normal value 0–1 mg) 1 year after right adrenalectomy. Adrenal CT scan demonstrated a 2.1 cm ovoid focus in the right suprarenal region, and functional imaging (131I meta-iodobenzylguanidine (MIBG scan) showed an avid uptake on the right frontal bone. Excision of the right adrenal bed and the right frontal bone tumour was performed, and metastatic pheochromocytoma was confirmed histologically. The patient achieved clinical and biochemical remission postoperatively and is currently receiving steroid and thyroxine replacement.

  • Adrenal disorders
  • Endocrine cancer

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Footnotes

  • Twitter @edrome14

  • Contributors EFH and NAN were responsible for drafting the text, sourcing and editing the clinical images, investigation results, drawing original diagrams and algorithms and critically revising the important intellectual content. EFH and NAN gave final approval of the manuscript.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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