Abstract
BackgroundThis study aims to introduce the diagnosis and surgical treatment of the rare disease multiple endocrine neoplasia type 2A (MEN 2A).MethodsThirteen cases of MEN 2A were diagnosed as medullary thyroid carcinoma (MTC) and pheochromocytoma by biochemical tests and imaging examination. They were treated by bilateral adrenal tumor excision or laparoscopic surgery.ResultsNine patients were treated by bilateral adrenal tumor excision and the remaining four were treated by laparoscopic surgery for pheochromocytoma. Ten patients were treated by total thyroidectomy and bilateral lymph nodes dissection and the remaining three were treated by unilateral thyroidectomy for MTC. Up to now, three patients have died of MTC distant metastasis.ConclusionsWe confirmed that MEN 2A can be diagnosed by biochemical tests and imaging examination when genetic testing is not available. Surgical excision is the predominant way to treat MEN 2A; pheochromocytoma should be excised at first when pheochromocytoma and MTC occur simultaneously.
Highlights
This study aims to introduce the diagnosis and surgical treatment of the rare disease multiple endocrine neoplasia type 2A (MEN 2A)
Multiple endocrine neoplasia type 2 (MEN 2) is divided into three subtypes: MEN 2A, MEN 2B, and familial medullary thyroid carcinoma [1]
MEN 2A is normally diagnosed by biochemical tests and imaging examinations [4,5]
Summary
This study aims to introduce the diagnosis and surgical treatment of the rare disease multiple endocrine neoplasia type 2A (MEN 2A). Multiple endocrine neoplasia type 2 (MEN 2) is an inherited syndrome which affects many endocrine glands. MEN 2A, known as Sipple syndrome, is a very rare disease. Genetic testing is currently the main method of MEN 2A diagnosis in developed countries. It has not yet been extensively spread in developing countries because of economic and technical limitations. Surgical excision is the predominant treatment of MEN 2A. The present study aims to describe this disease and to improve people’s awareness about the diagnosis and treatment of MEN 2A
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