PMID- 27311764
OWN - NLM
STAT- MEDLINE
DCOM- 20171023
LR  - 20230723
IS  - 1868-8500 (Electronic)
IS  - 1868-8497 (Print)
IS  - 1868-8497 (Linking)
VI  - 7
IP  - 4
DP  - 2016 Aug
TI  - Clinical Features, Treatments, and Outcomes of Patients with Thymic Carcinoids 
      and Multiple Endocrine Neoplasia Type 1 Syndrome at MD Anderson Cancer Center.
PG  - 279-87
LID - 10.1007/s12672-016-0269-y [doi]
AB  - Thymic carcinoids are rare neuroendocrine tumors that occur in 1-5 % of patients 
      with multiple endocrine neoplasia type 1 (MEN1) and are a major cause of 
      morbidity and mortality. The few published reports associate these tumors with 
      male sex and smoking. Our objective was to describe cases of these tumors treated 
      at our institution. We performed a retrospective chart review of all patients 
      diagnosed with MEN1 at our tertiary referral center from 1980 to 2014. Patients 
      with a histopathologic, fine-needle aspiration, or clinical diagnosis of a thymic 
      carcinoid were included. Two hundred ninety-one patients fulfilled the criteria 
      for a diagnosis of MEN1. Clinicopathologic characteristics, MEN1 genetic testing 
      results, treatments, and survival rates were analyzed. Nine patients had a thymic 
      carcinoid, six men (67 %) and three women (33 %). Six patients were non-smokers 
      (67 %). Two patients had synchronous (22 %) and eight patients (89 %) had 
      metachronous distant metastasis. The 10-year overall survival rate was 45 % 
      (lower 95 % upper 95 % CI 20-100 %). The 10-year disease-free survival rate was 
      42 % (lower 95 % upper 95 % CI 15-100 %). Five patients had MEN1 genetic testing, 
      and the genotypes of affected individuals were p.W341X, c.275_286delGCTTCACCGCCC, 
      p.R98X, c.1350+(1_11)del11, and partial duplication of exons 9 and 10. A higher 
      percentage of MEN1-related thymic carcinoids can occur in women and in 
      non-smokers than previously reported. Both novel and known mutations were present 
      in our cohort. Eighty nine percent of patients developed a metachronous 
      metastasis from the thymic carcinoid. Patients with MEN1 and thymic carcinoids 
      should be followed closely.
FAU - Christakis, Ioannis
AU  - Christakis I
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA.
FAU - Qiu, Wei
AU  - Qiu W
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA.
AD  - Department of Hepatobiliary Pancreatic Surgery, The First Hospital of Jilin 
      University, Changchun, Jilin, China.
FAU - Silva Figueroa, Angelica M
AU  - Silva Figueroa AM
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA.
FAU - Hyde, Samuel
AU  - Hyde S
AD  - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas 
      MD Anderson Cancer Center, Houston, TX, USA.
FAU - Cote, Gilbert J
AU  - Cote GJ
AD  - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas 
      MD Anderson Cancer Center, Houston, TX, USA.
FAU - Busaidy, Naifa L
AU  - Busaidy NL
AD  - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas 
      MD Anderson Cancer Center, Houston, TX, USA.
FAU - Williams, Michelle
AU  - Williams M
AD  - Department of Pathology, The University of Texas MD Anderson Cancer Center, 
      Houston, TX, USA.
FAU - Grubbs, Elizabeth
AU  - Grubbs E
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA.
FAU - Lee, Jeffrey E
AU  - Lee JE
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA.
FAU - Perrier, Nancy D
AU  - Perrier ND
AD  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer 
      Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, 
      USA. nperrier@mdanderson.org.
LA  - eng
GR  - P30 CA016672/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20160616
PL  - United States
TA  - Horm Cancer
JT  - Hormones & cancer
JID - 101518427
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adult
MH  - Carcinoid Tumor/*epidemiology/genetics
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*epidemiology/genetics
MH  - Neoplasms, Multiple Primary/epidemiology/genetics
MH  - Neoplasms, Second Primary/epidemiology/genetics
MH  - Prognosis
MH  - Proto-Oncogene Proteins/*genetics
MH  - Retrospective Studies
MH  - Risk Factors
MH  - Survival Analysis
MH  - Thymus Neoplasms/*epidemiology/genetics
MH  - Young Adult
PMC - PMC10355947
COIS- The authors declare that they have no conflicts of interest.
EDAT- 2016/06/18 06:00
MHDA- 2017/10/24 06:00
PMCR- 2016/06/16
CRDT- 2016/06/18 06:00
PHST- 2016/04/06 00:00 [received]
PHST- 2016/06/13 00:00 [accepted]
PHST- 2016/06/18 06:00 [entrez]
PHST- 2016/06/18 06:00 [pubmed]
PHST- 2017/10/24 06:00 [medline]
PHST- 2016/06/16 00:00 [pmc-release]
AID - 10.1007/s12672-016-0269-y [pii]
AID - 269 [pii]
AID - 10.1007/s12672-016-0269-y [doi]
PST - ppublish
SO  - Horm Cancer. 2016 Aug;7(4):279-87. doi: 10.1007/s12672-016-0269-y. Epub 2016 Jun 
      16.