Metastatic pheochromocytoma in MEN 2A: A rare association

BMJ Case Rep. 2018 Mar 28:2018:bcr2017222758. doi: 10.1136/bcr-2017-222758.

Abstract

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.

Keywords: adrenal disorders; endocrine cancer; thyroid disease.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Gland Neoplasms / pathology*
  • Adrenal Gland Neoplasms / surgery
  • Adrenal Glands / diagnostic imaging
  • Adrenal Glands / surgery
  • Antineoplastic Combined Chemotherapy Protocols
  • Diagnosis, Differential
  • Digestive System Neoplasms / diagnostic imaging
  • Digestive System Neoplasms / drug therapy
  • Digestive System Neoplasms / secondary*
  • Endocrine Gland Neoplasms / diagnostic imaging
  • Endocrine Gland Neoplasms / drug therapy
  • Endocrine Gland Neoplasms / secondary*
  • Female
  • Humans
  • Liver / diagnostic imaging
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 2a / pathology*
  • Multiple Endocrine Neoplasia Type 2a / surgery
  • Pancreas / diagnostic imaging
  • Pheochromocytoma / pathology*
  • Pheochromocytoma / surgery
  • Positron Emission Tomography Computed Tomography
  • Spleen / diagnostic imaging
  • Thyroid Gland / diagnostic imaging
  • Whole Body Imaging