Multimodal treatment for multifocal metastatic maxillary adenoid cystic carcinoma: A case report

Maxillary adenoid cystic carcinoma (ACC) is rare, with pleural and brain metastases representing an unprecedented presentation. The present case report illustrates the challenges and potential for prolonged survival in such an advanced stage of malignant disease. A 52-year-old male patient presented with a left maxillary mass, initially misdiagnosed as ameloblastoma; subsequently, it was confirmed as maxillary ACC (T4aN0M0, stage IVA). Following primary tumor resection and radiotherapy, the patient developed pleural (February 2021) and brain metastases (March 2022). A multimodal treatment strategy was used, involving aggressive surgical resection of the primary tumor, comprehensive radiotherapy, systemic chemotherapy regimens and adjuvant targeted molecular therapy. By using this sequential multimodal therapy, the patient achieved long-term survival exceeding 5 years. The present case report highlights the potential for favorable, prolonged survival even in the presence of multifocal metastatic maxillary ACC when managed with a persistent, sequential and tailored combination of modern therapeutic modalities.