Complete Response to Dual Immunotherapy in a Young Adult with Metastatic Alveolar Soft Part Sarcoma Enabled by a Drug Recovery Program in a Community Practice

Abstract

Alveolar soft part sarcoma (ASPS) is an extremely rare tumor that frequently occurs in adolescent and young adults (AYA). Survival is poor for patients with metastatic and/or relapsed disease not amenable to local control, and limited therapeutic options are available. A major barrier to cancer care in the United States AYA population is lack of access to coordinated care and appropriate therapies for those who lack insurance or who are underinsured. We report a 25-year-old unemployed, uninsured, single mother who presented with a 12.8 • 21 cm soft tissue thigh mass with heterogeneous avidity, max standardized uptake value of 9, with metastatic disease to the ipsilateral inguinal lymph nodes and to the bilateral lungs. After local control of the primary mass was obtained, a recently developed, comprehensive drug replacement program (DRP) was used to gain access to nivolumab, and after frank progression was noted, ipilimumab was added every 6 weeks. No biomarkers associated with response to immunotherapy were identified. After four cycles, a complete response was observed and patient remains disease free 36 months after beginning dual immunotherapy treatment. We obtained immunotherapy agents through a DRP and describe the development and the utility of this program in the community setting. Our report highlights both first documented sustained complete response to sequenced immunotherapy in an AYA with ASPS as well as a comprehensive DRP, which enabled access to therapy for our patient.

Successful treatment of advanced alveolar soft part sarcoma with camrelizumab combined with apatinib: a case report

“…Early studies have confirmed that PD-1/PD-L1 more efficient, and the immunosuppressive effect is longer, safer and more reliable (22)(23)(24). According to reports in the literature, ASPS PD-1 has a single-agent effective rate of 42-50% (5,25). In a study by Groisberg et al, four ASPS patients were treated with immunotherapy.…”

Anti-Angiogenic Agents in Management of Sarcoma Patients: Overview of Published Trials

“…ASPS are particularly vascularized. Some case-reports suggest that ASPS are sensitive to immunotherapy ( 72 ). Cediranib had been assessed in one single-arm phase 2 trials (with 2 cohorts) and then in a randomized phase 2 trial.…”

Complete response of metastatic alveolar soft part sarcoma in an adolescent female treated by combined immune checkpoint inhibitors

“…9 Recently, there have been two reported adult cases that showed positive responses after receiving the combination of nivolumab and ipilimumab. 12,13 The Korean adolescent presented in this report was successfully treated using combined ICPIs, despite the presence of several poor prognostic factors. To the best of our knowledge, this is the first report of an adolescent patient with metastatic ASPS who achieved CR with a combination of ICPIs.…”