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 is a rare malignancy with unique cellular structure and clinical and genetic characteristics. Most patients have no obvious clinical symptoms, and only a few develop respiratory symptoms. The typical histological characteristics are acinoid or organ-like structure. Immunofluorescence in situ hybridization suggests a rearrangement of the transcription factor E3 gene. Patients with primary acinar soft part sarcoma of the lung 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 who underwent targeted treatment combined with immunotherapy. Some of the lesions completely disappeared after treatment, indicating significant efficacy. The treatment benefit observed in the current case provides preliminary evidence that targeted therapy combined with immunotherapy maybe a safe and effective modality in patients with primary pulmonary ASPS, thus warranting further investigation.