Pheochromocytoma and Paraganglioma / PCPG (TCGA)

Overview

PCPG is the TCGA cohort identifier for pheochromocytoma and paraganglioma. The closest OncoTree equivalents are PHC (Pheochromocytoma) and PGNG (Paraganglioma). PCPG tumors have a high rate of hereditary predisposition (SDH subunits, RET, VHL, NF1, MAX) and frequent fusion-driven oncogenesis.

Cohorts in the corpus

  • TCGA PCPG cohort: included as one of 33 cancer types in the pan-cancer fusion landscape study; subset of the PanCancer Atlas (pcpg_tcga_pan_can_atlas_2018).

Recurrent alterations

  • Pan-cancer fusion study (9,624 TCGA samples) found PCPG has a median of 0 fusions per sample; TSG-partner fusion under-expression was highest in PCPG (38% of TSG-partner fusions), suggesting TSG inactivation via fusion is a dominant mechanism in PCPG PMID:29617662.

Subtypes

  • SDH-mutant cluster (pseudohypoxia): SDHB, SDHD, SDHC, VHL, EPAS1 mutations.
  • Kinase-signaling cluster: RET, NF1, TMEM127, MAX mutations.

Therapeutic landscape

No drug-specific findings for PCPG reported in the current corpus.

Sources

  • PMID:29617662 — Pan-cancer fusion landscape (Gao et al., 2018)

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