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CASE REPORT
Metastatic pheochromocytoma in MEN 2A: A rare association
  1. Rimesh Pal1,
  2. Ashu Rastogi1,
  3. Santosh Kumar2,
  4. Anil Bhansali1
  1. 1 Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  2. 2 Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  1. Correspondence to Dr Anil Bhansali, anilbhansaliendocrine{at}gmail.com

Summary

A 45-year-old woman was diagnosed as having multiple endocrine neoplasia type 2A in 2014. She had bilateral pheochromocytoma, medullary thyroid carcinoma and biopsy-proven cutaneous lichen amyloidosis in the interscapular area. She underwent bilateral adrenalectomy; following which, she achieved clinical and biochemical remission. She was planned for total thyroidectomy at a later date; however, she was lost to follow-up. She presented to us again in December 2016 with abdominal pain. Examination revealed hypertension with postural drop. Positron emission tomography scan showed Ga68 and fluorodeoxyglucose (FDG)-avid suprarenal, hepatic, peritoneal and mesenteric masses with abdominal lymph nodes. Twenty-four-hour urinary metanephrines/normetanephrines were elevated. Serum calcitonin was as high as it was 2-1/2 years ago. Ultrasonography-guided fine-needle aspiration cytology (FNAC) from the liver mass revealed neuroendocrine cells that did not stain for calcitonin. Hence, a diagnosis of metastatic pheochromocytoma was made. She underwent total thyroidectomy and was started on cyclophosphamide, vincristine, dacarbazine-based chemotherapy regimen.

  • adrenal disorders
  • thyroid disease
  • endocrine cancer
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Footnotes

  • Contributors RP prepared the manuscript. AR helped in the management of the patient. SK had been the operating surgeon. AB conceptualised the manuscript and provided overall guidance.

  • 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.

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