MEN 2A syndrome - Multiple endocrine neoplasia with autosomal dominant transmission

International Journal of Surgery Case Reports
Sergiu UngureanuRicharda Romanenco

Abstract

Multiple endocrine neoplasias (MEN) are rare inherited syndromes. MEN type 2 syndromes occur in 1:30000 individuals, and are reported in approximately 500-1000 families worldwide, the most frequent being MEN 2A (80%), followed by familial medullary thyroid carcinoma (15%) and MEN 2B (5%) (Marx and Wells, 2011; Dumitrache, 2012). The patient, a 20-years old with MEN 2A syndrome, which has been manifested by bilateral pheochromocytoma and medullary thyroid carcinoma. It was a familial form, having first degree relatives (mother) with pheochromocytoma. The patient underwent laparoscopic adrenalectomy on her left in 2015, laparoscopic adrenalectomy on the right in 2019 and total thyroidectomy in 2020 year. The postoperative evolution of this patient is favorable. She is satisfied with the received treatment and is being supervised by an endocrinologist, undergoing hormone replacement therapy. The case demonstrates the importance of the radical approach to MEN 2A syndrome from both a therapeutic and surgical point of view. If a pheochromocytoma is detected, adrenalectomy should be performed before thyroidectomy or other surgery to avoid intraoperative catecholamine release. Laparoscopy is the choice of approach in surgical treatment...Continue Reading

References

Jul 6, 2004·Current Treatment Options in Oncology·Michael E Gertner, Electron Kebebew
Sep 28, 2010·American Journal of Surgery·Avital Harari, William B Inabnet
Mar 22, 2013·Thyroid Research·Maria Alevizaki
Sep 14, 2013·The Journal of Clinical Endocrinology and Metabolism·Sonali ThosaniCamilo Jimenez
Mar 27, 2015·Thyroid : Official Journal of the American Thyroid Association·Samuel A WellsUNKNOWN American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma
Oct 21, 2018·International Journal of Surgery·Riaz A AghaUNKNOWN SCARE Group

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Methods Mentioned

BETA
Transfection
dissection
surgical resection
hormone replacement therapy

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