PMID- 33204258
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220418
IS  - 1687-8337 (Print)
IS  - 1687-8345 (Electronic)
IS  - 1687-8337 (Linking)
VI  - 2020
DP  - 2020
TI  - Clinical Characteristics and Management of Functional Pancreatic Neuroendocrine 
      Neoplasms: A Single Institution 20-Year Experience with 286 Patients.
PG  - 1030518
LID - 10.1155/2020/1030518 [doi]
LID - 1030518
AB  - BACKGROUND: Functional pancreatic neuroendocrine neoplasms (PanNENs) are very 
      rare disorders but have complex spectrum, including insulinoma, gastrinoma, 
      glucagonoma, somatostatinoma, and VIPoma. Patients with PanNENs usually present 
      with characteristic symptoms caused by corresponding hormone hypersecretion. It 
      has always been challenging in dealing with such rare but complicated disorders. 
      In this report, we analyzed the clinical characteristics of functional PanNENs in 
      a large cohort of Chinese patients and summarized our clinical experience in 
      diagnosis and treatment. METHODS: The retrospective analysis was performed in 
      patients with a definite diagnosis of functional PanNENs hospitalized in Chinese 
      PLA General Hospital between 2000 and 2020. The clinical characteristics, 
      surgical information, and pathological findings were extracted from their medical 
      records and were analyzed. RESULTS: Totally, 286 patients (gender: male 103 and 
      female 183; age: 45.55 +/- 15.23 years old) were diagnosed with definite functional 
      PanNENs. The most frequent functional PanNENs were insulinoma (266/286) followed 
      by glucagonoma (10/286), somatostatinoma (3/286), adrenocorticotropic hormone- 
      (ACTH-) producing tumor (3/286), gastrinoma (2/286), and VIPoma (2/286). Nine 
      patients were diagnosed with multiple endocrine neoplasia type 1 (MEN1) in which 
      all the associated functional PanNENs were insulinomas. The duration from 
      symptoms' onset to confirmed diagnosis was 3.67 +/- 4.28 years. Two hundred and 
      eighty patients with tumor localized in pancreatic or with limited metastasis 
      underwent surgery. The symptoms associated with hormonal oversecretion were 
      improved significantly after surgery. Five patients with unresectable metastases 
      or tumor recurrence after surgery were administrated with systemic chemotherapy 
      or other targeted therapies. With these various therapies, the symptoms were also 
      partially relieved. According to findings in pathological and immunochemical 
      examination, all the functional PanNENs were categorized into NEN-G1 (41.95%), 
      NEN-G2 (54.90%), NEN-G3 (3.15%), and NEC-G3 (0%). CONCLUSION: Patients with 
      suspected functional PanNENs should have a systematic endocrine examination at 
      diagnosis. Multidisciplinary collaborations are essential for precise diagnosis 
      and tumor localization. A successful surgery or other targeted therapies can 
      improve the prognosis of patients with such rare but complex disorders.
CI  - Copyright (c) 2020 Yuqing Qu et al.
FAU - Qu, Yuqing
AU  - Qu Y
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
AD  - Department of Endocrinology, Yantai Yuhuangding Hospital, Yantai 264000, Shandong 
      Province, China.
FAU - Li, Haoming
AU  - Li H
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
AD  - Department of Endocrinology, Shexian Hospital, Handan 056400, Hebei Province, 
      China.
FAU - Wang, Xianling
AU  - Wang X
AUID- ORCID: 0000-0001-7350-4101
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Chen, Yulong
AU  - Chen Y
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Guo, Qinghua
AU  - Guo Q
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Pei, Yu
AU  - Pei Y
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Du, Jin
AU  - Du J
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Dou, Jingtao
AU  - Dou J
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Ba, Jianming
AU  - Ba J
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Lv, Zhaohui
AU  - Lv Z
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
FAU - Mu, Yiming
AU  - Mu Y
AD  - Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
LA  - eng
PT  - Journal Article
DEP - 20201106
PL  - Egypt
TA  - Int J Endocrinol
JT  - International journal of endocrinology
JID - 101516376
PMC - PMC7665912
COIS- The authors declare that they have no conflicts of interest in this paper.
EDAT- 2020/11/19 06:00
MHDA- 2020/11/19 06:01
PMCR- 2020/11/06
CRDT- 2020/11/18 05:58
PHST- 2020/05/19 00:00 [received]
PHST- 2020/07/23 00:00 [revised]
PHST- 2020/10/15 00:00 [accepted]
PHST- 2020/11/18 05:58 [entrez]
PHST- 2020/11/19 06:00 [pubmed]
PHST- 2020/11/19 06:01 [medline]
PHST- 2020/11/06 00:00 [pmc-release]
AID - 10.1155/2020/1030518 [doi]
PST - epublish
SO  - Int J Endocrinol. 2020 Nov 6;2020:1030518. doi: 10.1155/2020/1030518. eCollection 
      2020.