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Adenoid Cystic Carcinoma — SCE Medical Oncology MCQ

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HardHead & Neck CancerAdenoid Cystic CarcinomaSCE Medical Oncology

A 60-year-old man with a salivary gland adenoid cystic carcinoma (ESC) of the parotid has progressive metastatic disease (lung metastases). He has been observed for 2 years but now has symptomatic progression. What systemic therapy options exist?

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Correct answer: E — Lenvatinib (multi-kinase TKI) has shown activity; chemotherapy options include cisplatin ± doxorubicin; MYB-NFIB fusion is the molecular hallmark but not yet targetable

Adenoid cystic carcinoma is characterised by the MYB-NFIB translocation. It has an indolent but relentless course with late distant metastases (especially lung). Systemic therapy options are limited: multi-kinase TKIs (lenvatinib — LEVANT study; axitinib) have shown the most activity (~12-15% ORR with prolonged disease stabilisation). Chemotherapy (cisplatin ± doxorubicin) has modest response rates (~15-20%). Clinical trials evaluating Notch pathway inhibitors and other novel approaches are ongoing.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/cancer