PMID- 28138639
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 2415-1289 (Print)
IS  - 2415-1289 (Electronic)
IS  - 2415-1289 (Linking)
VI  - 1
DP  - 2016
TI  - Metastatic multiple endocrine neoplasia type 1: report of one case.
PG  - 73
LID - 10.21037/tgh.2016.09.03 [doi]
LID - 73
AB  - A 46-year-old Chinese woman was admitted to our hospital because of presence of 
      space-occupying lesions in the liver for 2 months in April, 2015. She had a 
      family history of multiple endocrine neoplasia type 1 (MEN1) and physical 
      examination is unremarkable. Previously, she has performed surgery for primary 
      pituitary tumor in 2002 and radiosurgery for its recurrence. Around December 
      2014, she suffered from abdominal discomfort associated with regurgitation and 
      gastroscopy revealed "gastroduodenal ulcers", along with elevated gastrin. Next, 
      both magnetic resonance and computed tomography imaging showed multiple 
      intrahepatic lesions, the PET-CT unveiled uptake pattern of 18F-FDG in duodenum 
      and multiple intrahepatic lesions, resembling the appearance of metastatic 
      gastrinoma. The octreotide scan gave identical results. The parathyroid SPECT 
      scans showed abnormal concentrations of radioactivity in right parathyroid. She 
      also had an elevated serum chromogranin A (CgA) level. There was medical evidence 
      to show that she is metastatic MEN1. Although multiple liver metastases, they 
      were considered to be resectable after MDT consultation. The intraoperative 
      exploration found a 1.5 cm tumor on the surface of the tail of the pancreas, a 12 
      cm retroperitoneal lipoma and two liver metastases, sized 3.5 cm and 1.5 cm, 
      respectively. All these tumors were completely removed. For pancreatic tumor, 
      pathological findings met the diagnostic criteria of neuroendocrine tumor (NET) 
      (G2). Immunohistochemistry revealed neuroendocrine areas were diffusely positive 
      for ATRX, DAXX, SSR2, SSR5 and CgA. For lipoma, the FISH results were negative 
      for CDK4 and MDM2 genes. Postoperatively, adjuvant therapy with octreotide was 
      applied. This case suggested that, as for metastatic gastrinoma, a potentially 
      curative surgical debulking should be considered when a resection of complete or 
      more than 90% of metastatic lesions along with the primary site could be 
      achieved, which is helpful to control symptoms and delay the subsequent need for 
      therapy.
FAU - Huang, Cheng
AU  - Huang C
AD  - Department of Liver Surgery, Fudan University Zhongshan Hospital, Fudan 
      University Institute of Liver Cancer, Shanghai 200032, China.
FAU - Zhu, Xiaodong
AU  - Zhu X
AD  - Department of Liver Surgery, Fudan University Zhongshan Hospital, Fudan 
      University Institute of Liver Cancer, Shanghai 200032, China.
FAU - Han, Xu
AU  - Han X
AD  - Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai 
      200032, China.
FAU - Lou, Wenhui
AU  - Lou W
AD  - Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai 
      200032, China.
LA  - eng
PT  - Case Reports
DEP - 20160923
PL  - China
TA  - Transl Gastroenterol Hepatol
JT  - Translational gastroenterology and hepatology
JID - 101683450
PMC - PMC5244628
OTO - NOTNLM
OT  - Pancreatic neuroendocrine tumors (pancreatic NETs)
OT  - gastrinoma
OT  - metastatic multiple endocrine neoplasia type 1 (metastatic MEN1)
COIS- The authors have no conflicts of interest to declare.
EDAT- 2017/02/01 06:00
MHDA- 2017/02/01 06:01
PMCR- 2016/09/23
CRDT- 2017/02/01 06:00
PHST- 2016/07/25 00:00 [received]
PHST- 2016/09/02 00:00 [accepted]
PHST- 2017/02/01 06:00 [entrez]
PHST- 2017/02/01 06:00 [pubmed]
PHST- 2017/02/01 06:01 [medline]
PHST- 2016/09/23 00:00 [pmc-release]
AID - tgh-01-2016.09.03 [pii]
AID - 10.21037/tgh.2016.09.03 [doi]
PST - epublish
SO  - Transl Gastroenterol Hepatol. 2016 Sep 23;1:73. doi: 10.21037/tgh.2016.09.03. 
      eCollection 2016.