Advanced Epithelioid Malignant Peripheral Nerve Sheath Tumor Showing Complete Response to Combined Surgery and Chemotherapy: A Case Report

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare high-grade soft tissue sarcoma. The epithelioid variant accounts for 5% or less of MPNSTs; the clinical behavior of this variant is unclear. Reports of approximately 40 cases are available in the English literature; however, most reports addressed clinicopathological features rather than therapeutic procedures or clinical courses. We describe a case of a 62-year-old male with an epithelioid MPNST of the left foot. Multiple lung metastases developed after radical surgery on the primary lesion. The response to adjuvant chemotherapy including doxorubicin and ifosfamide was favorable, and thoracoscopic resection was subsequently performed on the remaining three metastases. No evidence of recurrence or metastasis was observed at the 12-month followup after the first operation. Further followup and chemotherapy may be required.

Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression

“…Several case reports have demonstrated the efficacy of neoadjuvant chemotherapy followed by surgery for advanced MPNST . However, no information is available regarding the efficacy of neoadjuvant chemotherapy for advanced intrathoracic MPNST.…”

“…Several case reports have demonstrated the efficacy of neoadjuvant chemotherapy followed by surgery for advanced MPNST. [10][11][12] However, no information is available regarding the efficacy of neoadjuvant chemotherapy for advanced intrathoracic MPNST. The resectability of the remaining tumor after chemotherapy may be an important issue in patients with locally advanced intrathoracic MPNST.…”

“…Most benign neurogenic tumors in the thorax develop in the posterior mediastinum, but MPNST has been reported to arise in various thoracic spaces, including the non‐posterior mediastinum and chest wall, as well as the posterior mediastinum . Because of the rarity of intrathoracic MPNST, the optimal method of diagnosis and the efficacy of chemotherapy for advanced intrathoracic MPNST are unknown …”

“…[4][5][6][7][8] Because of the rarity of intrathoracic MPNST, the optimal method of diagnosis and the efficacy of chemotherapy for advanced intrathoracic MPNST are unknown. [9][10][11][12] We encountered a case of locally advanced inoperable MPNST mainly involving the middle mediastinum. The patient was treated with doxorubicin plus ifosfamide chemotherapy, and a partial response was obtained.…”

Tumor targeted delivery of doxorubicin in malignant peripheral nerve sheath tumors

“…Doxorubicin is one of the anthracycline drugs used widely for treatment of several malignant solid and soft tissue cancers. Few case studies were reported where doxorubicin alone or in combination with other chemotherapeutic agents were used to treat MPNSTs with moderate efficacy[ 41 – 43 ]. Doxil, a pegylated liposomal doxorubicin, was demonstrated previously to be effective in metastatic soft tissue sarcoma patients [ 44 ].…”