Remarkable response to anti-PD1 immunotherapy in refractory metastatic high-grade myxofibrosarcoma patient: A case
1Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China.
Introduction:
Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. Immunotherapy targeting either programmed cell death protein 1 (PD-1) or programmed death ligand 1 (PD-L1) has recently shown a curative effect on multiple cancers including melanoma, non-small cell lung cancer, and renal cell carcinoma. Although the immunotherapy has been applied in sarcoma, there is little information about the efficiency to treat metastatic MFS.
Patient Concerns:
A 42-year-old male presented to the clinic with a mass in the left thigh. Mass resection and ligament replacement surgery were performed.
Diagnoses:
The patient was diagnosed as high-grade MFS (federation nationale des centres de lutte contre le cancer, Grade 3) with pulmonary metastasis.
Interventions:
In the past few years, he was treated with surgery, chemoradiotherapy, and Anlotinib (an angiogenesis inhibitor), but the metastatic lesion continued to progress. About 40% to 50% of tumor cells in his pulmonary tissues were showed positive PD-L1 expression and his tumor mutational burden was 215Muts. Thus, he received Camrelizumab (PD-1 inhibitor).
Outcomes:
Six months after the initiating immunotherapy of Camrelizumab, the size of pulmonary lesions showed marked shrinkage, indicating a partial response. After a follow-up of 18 months, the patient remained in good condition without progressive disease.
Conclusion:
This case described here demonstrated that immunotherapy of PD-1 inhibitor is a promising treatment option for refractory MFS with PD-L1 positive or tumor mutational burden -high, which could contribute to effective tumor response.
Insights
Immunotherapy with a programmed cell death protein 1 (PD-1) inhibitor showed promise for treating advanced myxofibrosarcoma (MFS). This approach led to significant tumor shrinkage and sustained remission in a patient with metastatic MFS.
Area of Science:
- Oncology
- Immunotherapy
- Sarcoma Research
Background:
- Myxofibrosarcoma (MFS) is an aggressive tumor with high metastatic potential.
- Programmed cell death protein 1 (PD-1) and programmed death ligand 1 (PD-L1) inhibitors have shown efficacy in various cancers.
- Limited data exists on immunotherapy's effectiveness in metastatic MFS.
Observation:
- A patient with high-grade MFS and pulmonary metastasis presented with disease progression despite prior treatments.
- Tumor tissues showed positive PD-L1 expression (40-50%) and high tumor mutational burden (215Muts).
- The patient received Camrelizumab, a PD-1 inhibitor.
Findings:
- Immunotherapy with Camrelizumab resulted in significant shrinkage of pulmonary lesions, indicating a partial response.
- The patient experienced sustained remission without progressive disease for 18 months.
- This suggests a positive response to PD-1 inhibition in refractory MFS.
Implications:
- PD-1 inhibitor immunotherapy is a potential treatment for refractory, metastatic MFS.
- Patients with PD-L1 positive or high tumor mutational burden MFS may benefit from this approach.
- Further research is warranted to explore immunotherapy's role in MFS treatment.


