Adenoid Cystic Carcinoma

Myb protein emerged as a prognostic indicator in the pathogenesis and clinical outcomes of patients with lacrimal gland ACC.

LA-R/M ACC has a low TMB profile overall. Median TMB was higher in NOTCH-activated ACC in both the NHS and MSK groups and TMB may have value in further stratifying patients with LA-R/M ACC.

Breast ACC typically presents as a hypervascular, highly suspicious mass. Despite frequent triple-negative profiles, it shows low proliferation, poor response to chemotherapy, and favorable prognosis.

P1/2, N=120, Not yet recruiting, National Cancer Institute (NCI)

Importantly, early clinical studies demonstrated that the EGFR-targeted ADC MRG003 and the TROP2-targeted ADC Sac-TMT showed notable efficacy in patients with refractory ACC and high expression of these targets. This pioneering study introduces a biomarker-based stratification system, categorizing ACC patients by histological subtype and target expression profiles, EGFR/TROP2 for non-solid ACC and B7-H4/Nectin-4 for solid ACC, to inform ADC therapy decisions.

P1/2, N=125, Recruiting, Remix Therapeutics | Trial completion date: Jun 2027 --> Sep 2027 | Trial primary completion date: Jun 2026 --> Sep 2027

P1, N=13, Completed, M.D. Anderson Cancer Center | Active, not recruiting --> Completed | Trial completion date: Dec 2028 --> Jun 2026 | Trial primary completion date: Dec 2028 --> Jun 2026

This case highlights the importance of recognizing this rare tumor in patients with prostatic mass and normal PSA, with diagnosis relying on characteristic histomorphology and immunophenotype.

To our knowledge, this represents one of the longest documented clinical courses of TACC with integrated genomic analysis. This case highlights the potential for TACC to remain indolent for decades before transitioning to late regional and systemic progression, underscoring the importance of long-term surveillance and comprehensive clinicopathological and molecular evaluation.

P=N/A, N=70, Recruiting, Eye & ENT Hospital of Fudan University

CEP is active in R/M ACC, and prior TKI exposure did not appear to reduce efficacy. Higher response rates in ACC-I tumors and the apparent PIK3CA-related survival deficit are exploratory observations that need prospective testing before they can guide treatment.

Although ACCB generally has a good prognosis, the treatment of this rare type of breast cancer remains challenging to ensure favorable outcomes with minimal sequelae. Initial staging and long-term follow-up using appropriate imaging modalities are essential for selecting tailored treatment strategies and detecting early relapses.