Salivary Carcinoma (SACA)

Overview

Salivary Carcinoma is the OncoTree umbrella for all malignant salivary gland tumors, sitting under HEAD_NECK. Key subtypes include ACYC (Adenoid Cystic Carcinoma), SDCA (Salivary Duct Carcinoma), ACCC (Acinic Cell Carcinoma), MUCC (Mucoepidermoid Carcinoma), and MASC (Mammary Analogue Secretory Carcinoma). These tumors are collectively rare and historically managed with surgery ± radiation; systemic options are limited, driving adoption of NGS-guided basket trials.

Cohorts in the corpus

  • hnc_mskcc_2016 — 56 salivary carcinomas (36 ACYC + 20 other salivary including SDCA, ACCC, MUCC, MASC) in 151 recurrent/metastatic head and neck tumors profiled by MSK-IMPACT 410-gene panel; dataset publicly available on cBioPortal PMID:27442865.

Recurrent alterations

  • NOTCH1 — activating mutations or amplifications in 22.2% of recurrent/metastatic ACYC (8.3-fold enriched vs primary ACYC); recurrent truncating events at serine 2467 PMID:27442865.
  • TERT — promoter mutations in 14% of ACYC — the first report of TERT promoter mutations in any salivary cancer PMID:27442865.
  • ETV6-NTRK3 — fusion identifying MASC within histologically diagnosed ACCC; enables TRK inhibitor therapy PMID:27442865.
  • ERBB2, AR, HRAS, PIK3CA — actionable alterations in SDCA and other salivary subtypes PMID:27442865.
  • PDGFRA, KDR, KIT — co-amplification in 4 ACYC patients treated with regorafenib PMID:27442865.
  • Salivary gland carcinoma showed responses to neratinib in the SUMMIT basket trial for HER2-mutant solid tumors, including kinase domain missense mutations (L755, V777), in the histology-agnostic solid tumor NOS cohort PMID:29420467

Subtypes

  • ACYC — largest salivary subgroup in the corpus (36 recurrent/metastatic cases); defined by NOTCH1 enrichment in advanced disease.
  • SDCA — ERBB2 and AR alterations; HER2-directed and androgen-deprivation therapy rationale.
  • ACCC — historically misclassified MASC cases; ETV6-NTRK3 testing critical.
  • MASC — defined by ETV6-NTRK3 fusion; TRK inhibitor responsive.
  • MUCC — HRAS mutations; farnesyl-transferase inhibitor candidates.

Therapeutic landscape

  • Basket trials are the dominant vehicle for therapeutic access in salivary carcinoma; 56 salivary patients contributed to the 14% NGS-guided therapy rate in the overall MSK-IMPACT cohort PMID:27442865.
  • TRK inhibitors — major/near-complete responses in MASC (ETV6-NTRK3) patients enrolled on basket trial PMID:27442865.
  • γ-Secretase inhibitors — candidate for NOTCH1-activating ACYC PMID:27442865.
  • FGFR inhibitors — for FGFR3-mutant ACYC PMID:27442865.
  • Regorafenib — used in 4 ACYC patients with PDGFRA/KDR/KIT co-amplification PMID:27442865.

Sources

  • PMID:27442865 — Morris et al. 2017 (JAMA Oncol). MSK-IMPACT of 56 salivary carcinomas (36 ACYC + 20 other); multiple actionable alteration classes; basket-trial enrollment enabling precision therapy.

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