Abstract
Primary pulmonary adenoid cystic carcinoma (PACC) is an exceedingly rare malignant lung tumor. We report an extremely rare case of a 38-year-old female. In 2013, a computed tomography (CT) scan suggested lung cancer in the left upper lobe, and a percutaneous biopsy confirmed PACC pathologically. She underwent surgical resection followed by postoperative adjuvant chemotherapy in the same year, with no recurrence during the 5-year post-operative follow-up. In December 2019, follow-up CT revealed bilateral renal metastases. Subsequently, in 2021, chest wall metastases developed. After sequential radiotherapy, chemotherapy, and combined immunotherapy, the chest wall mass was significantly reduced. During this period, multiple immune-related adverse events (irAEs) occurred. Upon progression of the renal metastases in 2024, ultrasound-guided ablation was performed. Subsequent re-evaluations showed essentially no viability in the bilateral renal tumors, and the chest wall mass remained stable. This patient was diagnosed with PACC in 2013. As of September 2025, her overall survival (OS) has exceeded 11 years. The successful management of this case is attributed to multimodal therapy. To date, no cases of PACC with concurrent bilateral renal and chest wall metastases have been reported, thus providing a valuable reference for the diagnosis and treatment of PACC.