Successful Management of Intrathoracic Phrenic Malignant Peripheral Nerve Sheath Tumor by Multimodal Treatment

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and can be associated with type 1 neurofibromatosis. They are rarely located at the mediastinum, most often at the posterior mediastinal compartment or the paravertebral gutters. MPNSTs of the anterior mediastinum arising from the phrenic nerve are exceptional. Their prognosis is poor due to the high potential of local tumor recurrence and metastasis. Complete surgical resection remains the only radical treatment. However, the outcomes after this treatment alone cannot be considered satisfactory. Preoperative chemotherapy could be an interesting therapeutic option for unresectable tumors. We report here the case of a 77-year-old woman with an MPNST located at the anterior mediastinum arising from the phrenic nerve. This tumor was successfully managed with multimodal therapy combining preoperative chemotherapy, complete surgery and adjuvant radiation.