Gynecologic large cell neuroendocrine carcinoma: A review
Large cell neuroendocrine carcinomas (LCNEC) are rare, aggressive high-grade neuroendocrine neoplasms within the neuroendocrine cell lineage spectrum. This manuscript provides a detailed review of published literature on LCNEC of gynecological origin. We performed a PubMed search for material availa...
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Online Access: | https://doi.org/10.1177/2036361320968401 |
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doaj-7e620fd89ce4478ab84a0c95d8cb6f002020-11-25T03:10:18ZengSAGE PublishingRare Tumors2036-36132020-10-011210.1177/2036361320968401Gynecologic large cell neuroendocrine carcinoma: A reviewGrant Burkeen0Aman Chauhan1Rohitashva Agrawal2Riva Raiker3Jill Kolesar4Lowell Anthony5B. Mark Evers6Susanne Arnold7Department of Internal Medicine, University of Kentucky, Lexington, KY, USAMarkey Cancer Center, University of Kentucky, Lexington, KY, USADivision of Medical Oncology, University of Kentucky, Lexington, KY, USADepartment of Internal Medicine, University of Kentucky, Lexington, KY, USADepartment of Pharmacy Practice and Science, University of Kentucky, Lexington, KY, USAMarkey Cancer Center, University of Kentucky, Lexington, KY, USADepartment of Surgery, University of Kentucky, Lexington, KY, USAMarkey Cancer Center, University of Kentucky, Lexington, KY, USALarge cell neuroendocrine carcinomas (LCNEC) are rare, aggressive high-grade neuroendocrine neoplasms within the neuroendocrine cell lineage spectrum. This manuscript provides a detailed review of published literature on LCNEC of gynecological origin. We performed a PubMed search for material available on gynecologic LCNEC. We analyzed 104 unique cases of gynecologic LCNECs, of which 45 were cervical primary, 45 were ovarian, 13 were uterine, and 1 was vaginal. A total of 45 cases of cervical LCNEC were identified with a median age of 36 years. Median overall survival was 16 months. We identified 45 ovarian LCNEC cases in the published literature with a median age of 54 years. Median overall survival was 8 months. 13 LCNEC cases of uterine origin were identified; 12 out of 13 were of endometrial origin and the median age was 71 years. The majority of patients presented with Stage III/IV disease (stages I–IV were 31%, 8%, 38%, and 23%, respectively). Gynecologic LCNEC is an aggressive malignancy. Our current understanding of the disease biology is very limited. Efforts are required to better understand the genomic and molecular characterizations of gynecological LCNEC. These efforts will elucidate the underlying oncogenic pathways and driver mutations as potential targets.https://doi.org/10.1177/2036361320968401 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Grant Burkeen Aman Chauhan Rohitashva Agrawal Riva Raiker Jill Kolesar Lowell Anthony B. Mark Evers Susanne Arnold |
spellingShingle |
Grant Burkeen Aman Chauhan Rohitashva Agrawal Riva Raiker Jill Kolesar Lowell Anthony B. Mark Evers Susanne Arnold Gynecologic large cell neuroendocrine carcinoma: A review Rare Tumors |
author_facet |
Grant Burkeen Aman Chauhan Rohitashva Agrawal Riva Raiker Jill Kolesar Lowell Anthony B. Mark Evers Susanne Arnold |
author_sort |
Grant Burkeen |
title |
Gynecologic large cell neuroendocrine carcinoma: A review |
title_short |
Gynecologic large cell neuroendocrine carcinoma: A review |
title_full |
Gynecologic large cell neuroendocrine carcinoma: A review |
title_fullStr |
Gynecologic large cell neuroendocrine carcinoma: A review |
title_full_unstemmed |
Gynecologic large cell neuroendocrine carcinoma: A review |
title_sort |
gynecologic large cell neuroendocrine carcinoma: a review |
publisher |
SAGE Publishing |
series |
Rare Tumors |
issn |
2036-3613 |
publishDate |
2020-10-01 |
description |
Large cell neuroendocrine carcinomas (LCNEC) are rare, aggressive high-grade neuroendocrine neoplasms within the neuroendocrine cell lineage spectrum. This manuscript provides a detailed review of published literature on LCNEC of gynecological origin. We performed a PubMed search for material available on gynecologic LCNEC. We analyzed 104 unique cases of gynecologic LCNECs, of which 45 were cervical primary, 45 were ovarian, 13 were uterine, and 1 was vaginal. A total of 45 cases of cervical LCNEC were identified with a median age of 36 years. Median overall survival was 16 months. We identified 45 ovarian LCNEC cases in the published literature with a median age of 54 years. Median overall survival was 8 months. 13 LCNEC cases of uterine origin were identified; 12 out of 13 were of endometrial origin and the median age was 71 years. The majority of patients presented with Stage III/IV disease (stages I–IV were 31%, 8%, 38%, and 23%, respectively). Gynecologic LCNEC is an aggressive malignancy. Our current understanding of the disease biology is very limited. Efforts are required to better understand the genomic and molecular characterizations of gynecological LCNEC. These efforts will elucidate the underlying oncogenic pathways and driver mutations as potential targets. |
url |
https://doi.org/10.1177/2036361320968401 |
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