High-Grade Neuroendocrine Carcinoma of the Ovary (HGONEC)
Overview
High-Grade Neuroendocrine Carcinoma of the Ovary (HGONEC) is a rare, aggressive malignancy under Ovarian Cancer (OOVC) in OncoTree. It may present as a pure neuroendocrine tumor or as part of a mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN).
Cohorts in the corpus
- pog570_bcgsc_2020 — 1 ovarian MiNEN patient (PN19; 60% adenocarcinoma / 40% neuroendocrine, classified as MNET/HGONEC) within 28-patient metastatic NEN WGTA cohort. PMID:24326773
Recurrent alterations
- The single ovarian MiNEN (PN19) in the POG cohort carried MYC amplification, KRAS gain-of-function mutation, SMAD4 loss, CDK8 and FLT1 amplifications, TP53 loss, and RB1 loss. It clustered with TCGA colorectal adenocarcinomas on transcriptome analysis — the only case not clustering with other NENs. High PD-L1 (CD274) expression and high DLL3 expression were identified as potential therapeutic targets. PMID:24326773
Subtypes
- MiNEN (Mixed neuroendocrine-non-neuroendocrine neoplasm) with adenocarcinoma component.
Therapeutic landscape
- Immune-checkpoint inhibitors (anti-PD-1/PD-L1) supported by high CD274/PDCD1 expression; DLL3 inhibitors supported by high DLL3 expression; no standard systemic therapy; patient died shortly after biopsy. PMID:24326773
Sources
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