title: Concomitant existence of pheochromocytoma in a patient with multiple endocrine neoplasia type 1.
abstract: BACKGROUND
  Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant inherited disorder that is classically characterized by the presence of neoplastic lesions of the parathyroid glands, the anterior pituitary gland, and the pancreas. However, MEN1 with concomitant pheochromocytoma is extremely rare.
  
  
  METHODS
  We report a case of MEN1 concomitant with pheochromocytoma. A 44-year-old Japanese man, who had undergone total parathyroidectomy due to primary hyperparathyroidism at the age of 18, was referred to our hospital with a complaint of a large abdominal tumor. He was diagnosed as having a giant insulinoma (maximum diameter 18 cm) in the pancreatic tail, five other non-functional neuroendocrine tumors in the pancreatic body and tail, multiple liver metastases of pancreatic neuroendocrine tumors, a pituitary prolactinoma, non-functional adrenal cortical adenomas, a pheochromocytoma in addition to a subcutaneous neurofibroma, and a cutaneous fibroma. The genetic screening revealed a deletion mutation at codons 83-84 in exon 2 of the MEN1 gene. He underwent distal pancreatectomy, splenectomy, cholecystectomy, right adrenalectomy, abdominal subcutaneous tumor excision, and cutaneous tumor biopsy for the purpose of tumor volume reduction. Extended right posterior segmentectomy with partial hepatectomy of S2, S3, and S8 was performed to resect residual tumors 9 months after the initial surgery. Although a newly formed liver metastasis was found 19 months after the hepatectomy, he is still alive 4 years and 4 months after the initial surgery.
  
  
  CONCLUSIONS
  We reported an extremely rare case of giant insulinoma and simultaneous occurrence of pheochromocytoma and adrenal cortical adenoma in the ipsilateral adrenal gland in a patient clinically and genetically diagnosed as having MEN1.
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan. r-okada@fmu.ac.jp.
authors: Ryo
authors: R
authors: Okada
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Tatsuo
authors: T
authors: Shimura
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Shigeyuki
authors: S
authors: Tsukida
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Jin
authors: J
authors: Ando
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Yasuhide
authors: Y
authors: Kofunato
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Tomoyuki
authors: T
authors: Momma
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Rei
authors: R
authors: Yashima
authors: Department of Surgery, Ohara General Hospital, Fukushima, Japan.
authors: Yoshihisa
authors: Y
authors: Koyama
authors: Department of Thyroid and Endocrinology, Fukushima Medical University, Fukushima, Japan.
authors: Shinichi
authors: S
authors: Suzuki
authors: Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
authors: Seiichi
authors: S
authors: Takenoshita
keywords: Giant insulinoma; Multiple endocrine neoplasia 1; Pheochromocytoma