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Composite paraganglioma‐ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with multiple endocrine neoplasia type 2B: A case report
Author(s) -
Yamasaki Mutsushi,
Sato Yoshiyasu,
Nomura Takeo,
Sato Fuminori,
Uchino Shinya,
Mimata Hiromitsu
Publication year - 2017
Publication title -
asian journal of endoscopic surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.372
H-Index - 18
eISSN - 1758-5910
pISSN - 1758-5902
DOI - 10.1111/ases.12332
Subject(s) - medicine , multiple endocrine neoplasia type 2 , pheochromocytoma , ganglioneuroma , multiple endocrine neoplasia , vanillylmandelic acid , thyroid carcinoma , medullary thyroid cancer , paraganglioma , medullary cavity , concomitant , thyroid , urology , pathology , neuroblastoma , homovanillic acid , biochemistry , chemistry , genetics , receptor , germline mutation , biology , serotonin , mutation , gene , cell culture
Abstract Multiple endocrine neoplasia type 2 (MEN2) is an autosomal‐dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2‐associated extra‐adrenal pheochromocytomas (paraganglioma) are rare. A 59‐year‐old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10‐mm left adrenal mass and a 30‐mm retroperitoneal mass. He had paroxysmal elevations in blood pressure and in urinary metanephrine and vanillylmandelic acid values. Laparoscopic excision of the left adrenal gland and retroperitoneal mass was performed. We experienced an extremely rare case of composite paraganglioma‐ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with MEN2B.