Adenoid Cystic Carcinoma

CNS involvement in ACC was associated with poor outcomes despite multimodal therapy. NOTCH1 alterations and leptomeningeal disease were frequent in this cohort, but these findings should be interpreted as hypothesis-generating given the small sample size and absence of a matched non-CNS comparator cohort.

EN1 is a highly sensitive marker for AdCC and has prognostic value. MYB is a useful complementary marker, particularly when polymorphous adenocarcinoma is a consideration.

We propose the term basaloid ASC mimicking AdCC (BAC MAC). Recognition of this new SCC variant is important to avoid misdiagnosis and mismanagement.

Given the advanced stage, the patient received a systemic combination of tislelizumab, cyclophosphamide, pegylated liposomal doxorubicin, and nedaplatin, initially combined with local intrapleural therapy. This case highlights a diagnostic pitfall in lung tumors exhibiting squamoid immunophenotypes and underscores the necessity of incorporating myoepithelial markers into the diagnostic workup. Furthermore, it provides a cautiously interpreted clinical observation of immune checkpoint inhibitor-based combination therapy in advanced PACC.

P2, N=33, Active, not recruiting, Memorial Sloan Kettering Cancer Center | Trial completion date: May 2026 --> May 2027 | Trial primary completion date: May 2026 --> May 2027

P2, N=798, Active, not recruiting, National Cancer Institute (NCI) | Trial primary completion date: May 2027 --> May 2026

P2, N=76, Not yet recruiting, Eye & ENT Hospital of Fudan University

RNA-seq identified 588 differentially expressed genes associated with MEOX1 overexpression, with enrichment in pathways including cytokine-cytokine receptor interaction, Toll-like receptor signaling, and G protein-coupled receptor signaling. Together, these findings indicate that enforced MEOX1 expression is associated with reduced malignant phenotypes in SACC models and with transcriptomic alterations in pathways related to immune response, G protein-coupled receptor signaling, and DNA damage response.

ADCT-601 demonstrates robust AXL expression linked to anti-tumor activity in preclinical models of ACC, establishing a proof of concept for targeting AXL in this rare cancer. These findings support clinical translation of AXL-targeting ADC as a novel biomarker-driven therapy for patients with ACC.

Next generation sequencing demonstrated a MYBL1::NFIB fusion. We thus further expand the spectrum of tumors that can transition from IDL.

This study described the global molecular signature of miRNAs in benign and malignant salivary gland neoplasms and provided preliminary evidence of miRNA dysregulation, suggesting a potential association between specific miRNA signatures and molecular pathways implicated in tumour progression.

Pulmonary involvement is most common, and prolonged surveillance is warranted. Molecular profiling may provide insight into disease behavior and inform future therapeutic strategies.