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.