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Metastatic pheochromocytoma in MEN 2A: A rare association
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Metastatic pheochromocytoma in MEN 2A: A rare association
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Journal Article
Metastatic pheochromocytoma in MEN 2A: A rare association
2018
Overview
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.
Publisher
BMJ Publishing Group Ltd,BMJ Publishing Group LTD,BMJ Publishing Group
Subject
/ Adrenal Gland Neoplasms - pathology
/ Adrenal Gland Neoplasms - surgery
/ Adrenal Glands - diagnostic imaging
/ Antineoplastic Combined Chemotherapy Protocols
/ 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
/ Multiple Endocrine Neoplasia Type 2a - pathology
/ Multiple Endocrine Neoplasia Type 2a - surgery
/ Pancreas - diagnostic imaging
/ Patients
/ Pheochromocytoma - pathology
/ Positron Emission Tomography Computed Tomography
/ Thyroid Gland - diagnostic imaging
/ Tumors
