Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are difficult to control despite aggressive treatment. In this report the authors describe the treatment and follow-up review of a patient with neurofibromatosis Type I who harbored a recurrent median nerve MPNST. The man underwent preoperative intraarterial and intravenous chemotherapy followed by additional surgery for gross-total removal and postoperative radiotherapy. Two courses of preoperative intraarterial cisplatin and intravenous Adriamycin produced significant tumor shrinkage. Gross-total removal of the remaining tumor without amputation of the arm was followed by fractionated radiotherapy (total minimum tumor dose 6485 cGy, maximal dose 6575 cGy). The patient is alive 9.5 years after treatment without evidence of tumor recurrence and with only focal median nerve functional deficits. A review of the patient's treatment is warranted to provide a description of a regimen that may be useful in the treatment of similar patients in the future.

Inhibition of Bcl-xL by ABT-737 enhances chemotherapy sensitivity in neurofibromatosis type 1-associated malignant peripheral nerve sheath tumor cells

“…A phase I clinical trial of R11577 in patients with NF1-related PNs revealed a limited efficacy of this drug in NF1 (12). In addition, as a clinical therapy for NF1-associated MPNSTs, a combination of chemotherapeutic agents, carboplatin/etoposide (13), cisplatin/adriamycin (14), and ifosfamide/doxorubicin (15,16), have been tested in patients with NF1-associated MPNSTs, after surgical resection and radiation treatments. Long-term investigations using a multimodel therapeutic strategy demonstrated that patients with NF1-associated MPNSTs showed a significantly lower response rate to chemotherapy compared to patients with MPNSTs not associated with NF1 (17), indicating that there are still many hurdles to overcome in chemotherapy for the NF1-associated MPNSTs.…”

A Novel Treatment Modality for Malignant Peripheral Nerve Sheath Tumor Using a Dual-Effect Liposome to Combine Photodynamic Therapy and Chemotherapy

“…The main concern in using cDDP is the possible platinum-induced peripheral neurotoxicity. It has been shown that the dose for intravenous chemotherapy of cDDP to treat human sarcoma or MPNST is 50-100 mg/m 2 [10,31]. In this study, the overall survival of mice bearing a large size of MPNST tumor significantly increased when the drug dose of cDDP escalated from 1.43 mg/kg to 4.07 mg/kg, which is around 4.3 to 12.3 mg/m 2 ( Figure 5), suggesting low risk of neurotoxicity.…”

“…It has been shown that administration of high-dose doxorubicin and cisplatin could inhibit the cell growth of metastatic soft-tissue sarcomas and invasive thymoma [9]. In an NF1 derived recurrent MPNST, pretreatment of cisplatin combined with doxorubicin before the large resection, followed by fractionated radiotherapy was suggested to be a useful regimen [10]. Recently, cisplatin combined with doxorubicin and ifosfamide was also suggested as an effective adjunctive chemotherapy for the treatment of sporadic MPNST [11].…”

Malignant Peripheral Nerve Sheath Tumor of Non-Neurofibromatosis Type I Metastasized to the Cerebrospinal Axis

“…Recently, the results of using adjuvant chemotherapy have been reported with limited successes. The Italian and German soft tissue sarcoma cooperative group reported an overall pediatric response rate of 45%, with the highest response noted within the ifosfamide group9,11,12).…”