PMID- 27572829
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160830
LR  - 20200930
IS  - 2198-7793 (Print)
IS  - 2198-7793 (Electronic)
IS  - 2198-7793 (Linking)
VI  - 2
IP  - 1
DP  - 2016 Dec
TI  - Concomitant existence of pheochromocytoma in a patient with multiple endocrine 
      neoplasia type 1.
PG  - 84
LID - 10.1186/s40792-016-0214-x [doi]
LID - 84
AB  - 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. 
      CASE REPORT: 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.
FAU - Okada, Ryo
AU  - Okada R
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan. r-okada@fmu.ac.jp.
FAU - Shimura, Tatsuo
AU  - Shimura T
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Tsukida, Shigeyuki
AU  - Tsukida S
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Ando, Jin
AU  - Ando J
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Kofunato, Yasuhide
AU  - Kofunato Y
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Momma, Tomoyuki
AU  - Momma T
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Yashima, Rei
AU  - Yashima R
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Koyama, Yoshihisa
AU  - Koyama Y
AD  - Department of Surgery, Ohara General Hospital, Fukushima, Japan.
FAU - Suzuki, Shinichi
AU  - Suzuki S
AD  - Department of Thyroid and Endocrinology, Fukushima Medical University, Fukushima, 
      Japan.
FAU - Takenoshita, Seiichi
AU  - Takenoshita S
AD  - Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, 
      Japan.
LA  - eng
PT  - Journal Article
DEP - 20160830
PL  - Germany
TA  - Surg Case Rep
JT  - Surgical case reports
JID - 101662125
PMC - PMC5005233
OTO - NOTNLM
OT  - Giant insulinoma
OT  - Multiple endocrine neoplasia 1
OT  - Pheochromocytoma
EDAT- 2016/08/31 06:00
MHDA- 2016/08/31 06:01
PMCR- 2016/08/30
CRDT- 2016/08/31 06:00
PHST- 2016/04/08 00:00 [received]
PHST- 2016/08/10 00:00 [accepted]
PHST- 2016/08/31 06:00 [entrez]
PHST- 2016/08/31 06:00 [pubmed]
PHST- 2016/08/31 06:01 [medline]
PHST- 2016/08/30 00:00 [pmc-release]
AID - 10.1186/s40792-016-0214-x [pii]
AID - 214 [pii]
AID - 10.1186/s40792-016-0214-x [doi]
PST - ppublish
SO  - Surg Case Rep. 2016 Dec;2(1):84. doi: 10.1186/s40792-016-0214-x. Epub 2016 Aug 
      30.