PMID- 33101196
OWN - NLM
STAT- MEDLINE
DCOM- 20210526
LR  - 20210526
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 11
DP  - 2020
TI  - Prevalence of Parathyroid Carcinoma and Atypical Parathyroid Neoplasms in 153 
      Patients With Multiple Endocrine Neoplasia Type 1: Case Series and Literature 
      Review.
PG  - 557050
LID - 10.3389/fendo.2020.557050 [doi]
LID - 557050
AB  - Purpose: The occurrence of parathyroid carcinoma (PC) and atypical parathyroid 
      neoplasm (APN) in multiple endocrine neoplasia type 1 (MEN1) is rare. The present 
      paper reports the cases of 3 MEN1-PC/APN patients at our center and discusses the 
      prevalence in a Chinese MEN1 cohort. Methods: This report is a retrospective 
      analysis of 153 MEN1-associated primary hyperparathyroidism (MEN1-HPT) patients 
      at our center, which included 3 MEN1-associated PC/APN (MEN1-PC/APN) patients. 
      The clinical manifestations, biochemical indices, pathological findings, and 
      therapy have been summarized along with the report of the genetic testing of the 
      3 patients. Results: Of the 153 MEN1-HPT patients, 1 (0.7%) was 
      histopathologically diagnosed with PC and 2 (1.3%) with APN. Three heterozygous 
      mutations were identified in the 3 MEN1-PC/APN patients (c.917 T > G, c.431T > C, 
      and c.549 G > C). The cumulative findings of 3 cases with 18 previously reported 
      MEN1-PC/APN cases revealed that the mean serum calcium (Ca) level was 3.15 +/- 0.44 
      mmol/L and the median parathyroid hormone (PTH) level was 327 pg/mL (214.1, 
      673.1), both of which were significantly higher as compared to the respective 
      levels in non-PC/APN MEN1 patients at our center [Ca: 2.78 mmol/L [2.61, 2.88], 
      PTH: 185.5 pg/mL [108.3, 297.0]; P = 0.0003, 0.0034, respectively]. Conclusion: 
      MEN 1-PC/APN is a rare disease, with a prevalence of only 2.0% among the MEN1-HPT 
      cohort at our center. The affected patients recorded higher serum Ca level and 
      PTH levels than those with MEN1-associated benign tumors. However, the diagnosis 
      of MEN1-PC/APN is based upon pathology most of the times.
CI  - Copyright (c) 2020 Song, Yang, Liu, Nie, Jiang, Li, Xia, Wang and Xing.
FAU - Song, An
AU  - Song A
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Yang, Yi
AU  - Yang Y
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Liu, Shuzhong
AU  - Liu S
AD  - Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Peking 
      Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
FAU - Nie, Min
AU  - Nie M
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Jiang, Yan
AU  - Jiang Y
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Li, Mei
AU  - Li M
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Xia, Weibo
AU  - Xia W
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Wang, Ou
AU  - Wang O
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
FAU - Xing, Xiaoping
AU  - Xing X
AD  - Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, 
      Peking Union Medical College, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Sciences, Beijing, China.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20200930
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
RN  - SY7Q814VUP (Calcium)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Calcium/blood
MH  - Child
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications/genetics
MH  - Mutation
MH  - Parathyroid Neoplasms/*epidemiology/genetics
MH  - Prevalence
MH  - Retrospective Studies
MH  - Young Adult
PMC - PMC7556219
OTO - NOTNLM
OT  - Chinese population
OT  - atypical parathyroid neoplasms
OT  - multiple endocrine neoplasia type 1
OT  - parathyroid carcinoma
OT  - prevalence
EDAT- 2020/10/27 06:00
MHDA- 2021/05/27 06:00
PMCR- 2020/01/01
CRDT- 2020/10/26 05:21
PHST- 2020/06/10 00:00 [received]
PHST- 2020/08/20 00:00 [accepted]
PHST- 2020/10/26 05:21 [entrez]
PHST- 2020/10/27 06:00 [pubmed]
PHST- 2021/05/27 06:00 [medline]
PHST- 2020/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2020.557050 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2020 Sep 30;11:557050. doi: 
      10.3389/fendo.2020.557050. eCollection 2020.