PMID- 33334800
OWN - NLM
STAT- MEDLINE
DCOM- 20210611
LR  - 20240102
IS  - 1550-8080 (Electronic)
IS  - 0091-7370 (Linking)
VI  - 50
IP  - 6
DP  - 2020 Nov
TI  - Thymic Carcinoid as the First Manifestation of Multiple Endocrine Neoplasia Type 
      1 Syndrome: A Case Report and Review of the Literature.
PG  - 825-833
AB  - The present study reported a rare case with thymic carcinoid as the first 
      manifestation of multiple endocrine neoplasia type 1 (MEN1) syndrome, which 
      presented with gene mutations of the MEN1 and glucokinase regulatory protein 
      (GCKR). In this report, a 40-year-old male was diagnosed as MEN1 syndrome with 
      thymic carcinoid, pancreatic cancer, hyperparathyroidism, and insulinoma with 
      intrahepatic metastasis. Genetic testing showed the mutations of the MEN1 (c.378 
      G>A, p. Trp126*) in the patient, his children and two sisters, and GCKR 
      (c.151C>T, p. Arg51*) gene in the patient and his children. The pathological 
      examination showed that neuroendocrine tumor (NET) of the pancreas was 
      characterized as 6 mitoses per 10 high-power fields (HPF), infiltration of 
      adipose tissue, no intravascular tumor thrombus and nerve infiltration. In 
      addition, NET of the liver was characterized as 4 mitoses per 10 HPF, no 
      intravascular tumor thrombus and nerve infiltration. Immunohistochemical staining 
      showed Ckpan (+), Syn (+), CgA (+), CD 56 (+), PGP 9.5 (+), and Ki67-positive 
      cells (8-10%) in NET. Therefore, we suggested that genetic testing in family 
      members of MEN1 patient, which may be helpful for early diagnosis.
CI  - (c) 2020 by the Association of Clinical Scientists, Inc.
FAU - Zheng, Meiling
AU  - Zheng M
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China.
FAU - Chen, Shuchun
AU  - Chen S
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China 
      chenshuchunwork88@163.com renluping1122@163.com.
FAU - Qi, Cuijuan
AU  - Qi C
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China.
FAU - Ren, Luping
AU  - Ren L
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China 
      chenshuchunwork88@163.com renluping1122@163.com.
FAU - Zhen, Yunfeng
AU  - Zhen Y
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China.
FAU - Yu, Xian
AU  - Yu X
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China.
FAU - Wei, Limin
AU  - Wei L
AD  - Department of Endocrinology, Hebei General Hospital, Shijiazhaung, China.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Ann Clin Lab Sci
JT  - Annals of clinical and laboratory science
JID - 0410247
RN  - 0 (Adaptor Proteins, Signal Transducing)
RN  - 0 (GCKR protein, human)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adaptor Proteins, Signal Transducing/genetics
MH  - Adult
MH  - Carcinoid Tumor/genetics/*pathology
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia/diagnosis/genetics/metabolism
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/genetics/*metabolism
MH  - Neuroendocrine Tumors
MH  - Pancreatic Neoplasms
MH  - Proto-Oncogene Proteins/genetics
MH  - Thymoma/diagnosis/metabolism/pathology
OTO - NOTNLM
OT  - Gene mutation
OT  - Hyperparathyroidism
OT  - Multiple endocrine neoplasia type 1 (MEN1)
OT  - Pancreatic cancer
OT  - Prolactinoma
OT  - Thymic carcinoid
EDAT- 2020/12/19 06:00
MHDA- 2021/06/12 06:00
CRDT- 2020/12/18 05:40
PHST- 2020/12/18 05:40 [entrez]
PHST- 2020/12/19 06:00 [pubmed]
PHST- 2021/06/12 06:00 [medline]
AID - 50/6/825 [pii]
PST - ppublish
SO  - Ann Clin Lab Sci. 2020 Nov;50(6):825-833.