Rexin‑G®, a tumor‑targeted retrovector for malignant peripheral nerve sheath tumor: A case report
- Seth Kim
- Noah Federman
- Erlinda M. Gordon
- Frederick L. Hall
- Sant P. Chawla
Affiliations: Sarcoma Oncology Center/Cancer Center of Southern California, Santa Monica, CA 90403, USA, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Mattel Children's Hospital at UCLA, Los Angeles, CA 90095, USA, Counterpoint Biomedica LLC, Santa Monica, CA 90403, USA
- Published online on: April 28, 2017 https://doi.org/10.3892/mco.2017.1231
Copyright: © Kim
et al. This is an open access article distributed under the
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Soft tissue sarcoma is a rare neoplasm of mesenchymal origin, accounting for only ~1% of all adult cancers and consisting of 75 histological subtypes. In the present report, the unique case of a 14 year‑old female with metastatic malignant peripheral nerve sheath tumor (formerly, malignant melanotic schwannoma) of the parotid gland, who experienced a durable response and sustained tumor control with Rexin‑G®, a tumor‑targeted retroviral expression vector encoding an anti‑cyclin G1 construct, is described. Post‑parotidectomy, and prior to the administration of Rexin‑G®, the patient received various chemotherapy regimens, including doxorubicin, ifosfamide, temozolomide, sorafenib, and an immunological therapy with interleukin‑2, which only resulted in the further progression of lung metastases. The patient subsequently participated in a Phase 1/2 gene therapy study, during which she received intravenous Rexin‑G® as monotherapy for two years with minimal drug‑associated adverse events. Currently, the patient has no evidence of active disease 9 years after commencing the Rexin‑G® treatment, and with no additional anti‑cancer therapy. In conclusion, Rexin‑G® may be a viable therapeutic option for malignant peripheral nerve sheath tumors, and should be further investigated in prospective histology‑specific clinical trials for this type, and possibly other types, of chemotherapy‑resistant sarcoma.