title: Composite paraganglioma-ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with multiple endocrine neoplasia type 2B: A case report.
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.
authors.affiliation: Department of Urology, Oita University Faculty of Medicine, Yufu, Japan.
authors.forename: Mutsushi
authors.initials: M
authors.lastname: Yamasaki
authors.affiliation: Department of Urology, Oita University Faculty of Medicine, Yufu, Japan.
authors.forename: Yoshiyasu
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Department of Urology, Oita University Faculty of Medicine, Yufu, Japan.
authors.forename: Takeo
authors.initials: T
authors.lastname: Nomura
authors.affiliation: Department of Urology, Oita University Faculty of Medicine, Yufu, Japan.
authors.forename: Fuminori
authors.initials: F
authors.lastname: Sato
authors.affiliation: Noguchi Thyroid Clinic and Hospital Foundation, Beppu, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Uchino
authors.affiliation: Department of Urology, Oita University Faculty of Medicine, Yufu, Japan.
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Mimata
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000556:secondary* / Q000601:surgery; D018278:Carcinoma, Neuroendocrine / Q000473:pathology*; D005729:Ganglioneuroma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D018814:Multiple Endocrine Neoplasia Type 2b / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D018193:Neoplasms, Complex and Mixed / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D010235:Paraganglioma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D013964:Thyroid Neoplasms / Q000473:pathology*
keywords: Ganglioneuroma; multiple endocrine neoplasia type 2B; paraganglioma