PMID- 31727021
OWN - NLM
STAT- MEDLINE
DCOM- 20200326
LR  - 20200326
IS  - 1471-2407 (Electronic)
IS  - 1471-2407 (Linking)
VI  - 19
IP  - 1
DP  - 2019 Nov 14
TI  - Atypical ovarian carcinoid tumor with widespread skeletal metastases: a case 
      report of multiple endocrine neoplasia type 1 in a young woman.
PG  - 1107
LID - 10.1186/s12885-019-6332-7 [doi]
LID - 1107
AB  - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal 
      dominant inherited condition affecting multiple endocrine organs, resulting in 
      significant morbidity and decreased life expectancy. Early tumor identification 
      allows for timely patient management, reduces morbidity, and improves disease 
      outcomes. Patients with MEN1 typically present with primary hyperparathyroidism 
      caused by multiple parathyroid tumors, however, thymic and bronchial carcinoid 
      tumors are also less common manifestations. MEN1-related neuroendocrine tumors 
      often show hematogenous metastasis, with the liver being the most common 
      metastatic site. Skeletal metastases from neuroendocrine tumors are extremely 
      rare. As few as 50 case reports were identified in a recently published 
      literature review on skeletal metastases from carcinoid tumors. To our knowledge, 
      studies related to MEN1 have not been previously conducted. CASE PRESENTATION: We 
      present a case of MEN1-related atypical ovarian carcinoid presenting as the first 
      disease manifestation in a 30-year old woman. After two years, another atypical 
      carcinoid was incidentally diagnosed in the contralateral ovary during a 
      caesarean section. Syndromic MEN1 was not diagnosed clinically despite her young 
      age and bilateral involvement. The patient remained disease-free for two years 
      without further adjuvant treatment prior to clinic presentation with complaints 
      of chest discomfort and body pain. Radiologic and pathologic investigations 
      identified multifocal simultaneous neuroendocrine tumors involving the 
      parathyroid, thymus, pancreas, and adrenal glands, in addition to multiple other 
      metastatic sites. The findings ultimately resulted in the patient being diagnosed 
      with MEN1. CONCLUSIONS: This extremely rare case emphasizes that ovarian 
      carcinoids, especially when bilateral, could be the initial manifestation of 
      MEN1. The significance of this differential diagnosis was highlighted by the 
      subsequent detection of widespread skeletal metastasis resulting from the 
      carcinoid tumors. A low threshold of suspicion, systemic diagnostic work-up, and 
      regular follow-up are of utmost importance to timely diagnosis of MEN1.
FAU - Lou, Lei
AU  - Lou L
AD  - Department of Pathology, The Second Hospital of Hebei Medical University, 
      Shijiazhuang City, Hebei Province, 050000, People's Republic of China.
FAU - Zhou, Lixia
AU  - Zhou L
AD  - Department of Radiology, The Second Hospital of Hebei Medical University, 
      Shijiazhuang City, Hebei Province, 050000, People's Republic of China.
FAU - Wang, Wenyan
AU  - Wang W
AD  - Department of Radiotherapy, The Second Hospital of Hebei Medical University, 
      Shijiazhuang City, Hebei Province, 050000, People's Republic of China.
FAU - Li, Huina
AU  - Li H
AD  - Department of Pathology, Hebei Maternity and child Healthcare Hospital, 
      Shijiazhuang City, Hebei Province, 050000, People's Republic of China.
FAU - Li, Yuehong
AU  - Li Y
AUID- ORCID: 0000-0003-0737-3646
AD  - Department of Pathology, The Second Hospital of Hebei Medical University, 
      Shijiazhuang City, Hebei Province, 050000, People's Republic of China. 
      liyuehong1993@126.com.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20191114
PL  - England
TA  - BMC Cancer
JT  - BMC cancer
JID - 100967800
SB  - IM
MH  - Adult
MH  - Carcinoid Tumor/*diagnosis/diagnostic imaging/pathology
MH  - *Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Multiple Endocrine Neoplasia/*diagnosis/diagnostic imaging/pathology
MH  - Ovarian Neoplasms/*diagnosis/diagnostic imaging/pathology
MH  - Ovary/diagnostic imaging/pathology
PMC - PMC6857273
OTO - NOTNLM
OT  - Carcinoid tumor
OT  - MEN1
OT  - Multiple endocrine neoplasia type 1 syndrome
OT  - Neuroendocrine tumors
OT  - Primary hyperparathyroidism
OT  - Skeletal metastasis
COIS- The authors declare that they have no competing interests.
EDAT- 2019/11/16 06:00
MHDA- 2020/03/27 06:00
PMCR- 2019/11/14
CRDT- 2019/11/16 06:00
PHST- 2019/08/25 00:00 [received]
PHST- 2019/11/05 00:00 [accepted]
PHST- 2019/11/16 06:00 [entrez]
PHST- 2019/11/16 06:00 [pubmed]
PHST- 2020/03/27 06:00 [medline]
PHST- 2019/11/14 00:00 [pmc-release]
AID - 10.1186/s12885-019-6332-7 [pii]
AID - 6332 [pii]
AID - 10.1186/s12885-019-6332-7 [doi]
PST - epublish
SO  - BMC Cancer. 2019 Nov 14;19(1):1107. doi: 10.1186/s12885-019-6332-7.