ESRRG

Overview

ESRRG (Estrogen Related Receptor Gamma) is a nuclear receptor transcription factor. In cancer genomics, ESRRG has been identified as a noncanonical MYB fusion partner in sinonasal adenoid cystic carcinoma, and separately as the recipient in a novel ESRRG::DNM3 fusion in the same tumor type.

Alterations observed in the corpus

  • Novel ESRRGex3::DNM3ex14 fusion detected in 1 maxillary sinus metatypical adenoid cystic carcinoma infiltrating the maxilla and arising in respiratory epithelial adenomatoid hamartoma; not previously described in adenoid cystic carcinoma PMID:24418857
  • ESRRG also identified as a noncanonical MYB fusion partner in sinonasal adenoid cystic carcinoma; diagnostic workup with FISH recommended when canonical MYB::NFIB fusion is absent PMID:24418857

Cancer types (linked)

  • Sinonasal adenoid cystic carcinoma: novel ESRRG::DNM3 fusion in a single metatypical case; ESRRG rearrangement detected by FISH PMID:24418857

Co-occurrence and mutual exclusivity

  • ESRRG::DNM3 and noncanonical MYB fusions co-characterize a metatypical histological subset of sinonasal adenoid cystic carcinoma PMID:24418857

Therapeutic relevance

  • No direct therapeutic targeting reported in corpus; pairing RNA-seq panels with FISH for ESRRG is recommended for diagnostic workup of metatypical sinonasal adenoid cystic carcinoma PMID:24418857

Open questions

  • ESRRG::DNM3 was observed in only 1 case; generalizability of the metatypical-histology association requires larger cohorts PMID:24418857

Sources

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