Treatment of a Malignant Peripheral Nerve Sheath Tumor

Abstract

Background: Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor deriving from or differentiating into cells of the peripheral nerve sheaths, with a high risk of local recurrence and distant metastasis. Despite aggressive surgery and adjuvant therapy, the prognosis of MPNST is poor with reported 5-year survival rates of about 50%. Case Report: We report a case of a patient with MPNST of the neck, who developed multiple thoracic metastases after a 14-year disease-free interval. The patient underwent a total of 4 thoracic surgeries in the last 3 years because of local recurrence with tumor invasion to the chest wall and pericardium. Aggressive surgical intervention and adjuvant radiotherapy resulted in good outcome and long-term survival. Conclusion: Although MPNST is an aggressive carcinoma with poor survival, aggressive surgical intervention and radiotherapy resulted in good survival.

RETRACTED ARTICLE: Genomic amplification and high expression of EGFR are key targetable oncogenic events in malignant peripheral nerve sheath tumor

“…Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant sarcomas derived from the neural crest [1,2]. The relative rarity of MPNST and the lack of any specific diagnostic, radiologic, or pathologic signature pose considerable management challenges for the disease.…”

“…The relative rarity of MPNST and the lack of any specific diagnostic, radiologic, or pathologic signature pose considerable management challenges for the disease. Even with multidisciplinary treatment, the prognosis for patients with MPNST is still very poor [1,2]. The dismal outcome highlights the necessity of finding new therapeutic methods to benefit patients with this aggressive sarcoma [1-3].…”

Genomic and Molecular Characterization of Malignant Peripheral Nerve Sheath Tumor Identifies the IGF1R Pathway as a Primary Target for Treatment

“…Malignant peripheral nerve sheath tumors (MPNSTs), a subtype of soft-tissue sarcomas of neural crest origin (1), are highly malignant and account for approximately 5–10% of all soft-tissue sarcomas (2). Currently, the 5-year survival rates of MPNST patients are still only 30–50%, even with multidisciplinary treatments such as aggressive surgery, high-dose adjuvant chemotherapy, and radiotherapy (1).…”

“…Currently, the 5-year survival rates of MPNST patients are still only 30–50%, even with multidisciplinary treatments such as aggressive surgery, high-dose adjuvant chemotherapy, and radiotherapy (1). The dismal outcome not only points to the urgent need to establish better therapeutic strategies for patients harboring MPNSTs but also highlights the importance of exploring the genomic basis of the disease to identify recurrent oncogenic events for targeted therapy.…”

PET Imaging of Peripheral Nerve Tumors

“…1,2 Malignant peripheral nerve sheath tumors (MPNST) are the sixth most frequent soft tissue sarcoma (STS), consisting of 5% to 10% of cases. [3][4][5] Approximately 50% of all MPNST develop sporadically, whereas the other 50% are associated with neurofibromatosis type 1 (NF1). [6][7][8] Patients with NF1 have an 8% to 13% lifetime risk of developing MPNST, with an incidence of 1:3500, compared with the incidence of 1:100,000 among the general population.…”