Acinic Cell Carcinoma (ACCC)

Overview

Acinic Cell Carcinoma is a low- to intermediate-grade salivary gland malignancy sitting under SACA in OncoTree. A clinically important finding is that a subset of tumors histologically diagnosed as acinic cell carcinoma harbor ETV6-NTRK3 fusions and are better classified as Mammary Analogue Secretory Carcinoma (MASC), with implications for TRK-inhibitor sensitivity.

Cohorts in the corpus

• hnc_mskcc_2016 — included as part of the salivary carcinoma cases in 151 recurrent/metastatic head and neck tumors profiled by MSK-IMPACT 410-gene panel PMID:27442865.

Recurrent alterations

• ETV6-NTRK3 fusion — identified in 2 tumors initially diagnosed as acinic cell carcinoma by MSK-IMPACT NGS; these were reclassified as MASC (Mammary Analogue Secretory Carcinoma); both patients subsequently enrolled on a TRK inhibitor basket study with major or near-complete responses PMID:27442865.

Subtypes

• ACCC overlaps histologically with MASC; ETV6-NTRK3 fusion testing is warranted in all cases to enable correct classification and TRK-inhibitor access PMID:27442865.

Therapeutic landscape

• ETV6-NTRK3 fusions warrant TRK inhibitor evaluation — the reclassification of ETV6-NTRK3-positive acinic cell carcinomas as MASC enabled enrollment on TRK inhibitor basket trials with dramatic responses PMID:27442865.

Sources

• PMID:27442865 — Morris et al. 2017 (JAMA Oncol). ETV6-NTRK3 fusions identified in 2 ACCC cases, reclassified as MASC; dramatic TRK inhibitor responses on basket trial.

This page was processed by crosslinker on 2026-05-14.