Combined immunotherapy and targeted treatment for primary alveolar soft part sarcoma of the lung: case report and literature review.

ABSTRACT

Primary acinar soft part sarcoma of the lung (ASPS) is a rare malignancy with unique cellular structure and clinical and genetic characteristics. Most patients do not exhibit clear clinical symptoms, with only a few developing respiratory symptoms. The typical histological characteristics are acinoid or organ-like structures. Immunofluorescence in situ hybridization suggests a rearrangement of the transcription factor E3 gene. Patients respond poorly to chemotherapy and are, thus, primarily treated with surgery and targeted therapy. We report herein a unique case of primary alveolar soft part sarcoma of the lung. The patient was a 24-year-old man with metastases to multiple organs, such as the brain, lungs, pancreas, and liver. The craniocerebral lesions attained partial remission after whole-brain radiotherapy and targeted combined immunotherapy, and other distant metastases completely disappeared after targeted combined immunotherapy (anlotinib and camrelizumab), indicating significant treatment efficacy. Anlotinib is an oral multi-target tyrosine kinase inhibitor (TKI) that exerts its anti-tumor effects by acting on various kinases. Camrelizumab is a humanized immunoglobulin G4 monoclonal antibody that can target PD-1 to block the interaction between PD-L1 and programmed death ligand 2, ultimately causing an anti-tumor effect. This is the first report of successful use of anlotinib combined with camrelizumab in the treatment of advanced primary ASPS. The treatment benefit provides preliminary evidence that targeted therapy, combined with immunotherapy, may be a safe and effective approach to treat primary pulmonary ASPS patients, thus warranting further investigation.