How to Treat Metastatic Malignant Triton Tumor in an Adolescent

Which treatment strategy for MTT would be most effective and provide the best possible prognosis in this case? * A. Surgery * B. Chemotherapy * C. Chemotherapy and radiotherapy * D. Combination chemotherapy with surgery and/or radiation therapy Advertisement CORRECT ANSWER: * D. Combination chemotherapy with surgery and/or radiation therapy Discussion Malignant peripheral nerve sheath tumor (MPNST), also known as malignant schwannoma or neurogenic sarcoma, is a rare form of soft tissue sarcoma (STS) that develops from Schwann cells or pluripotent cells of the neural crest, constituting peripheral nerve branches or sheaths of peripheral nerve fibers.1 MPNST represents approximately 5% of all STS.2 In turn, MTT is a histological subtype of MPNST, in which malignant Schwann cells coexist with malignant rhabdomyoblasts.2,3 MTT accounts for less than 10% of MPNST.4,5 MTT is a highly aggressive tumor that most often affects patients aged less than 35 years, although it is very rare in pediatric patients.6 The prognosis is poor; 5-year survival rate among children ranges between 5% and 20%.2 The disease occurs equally among males and females.7 MTT may originate in different parts of the body, affecting extremities, head, neck, or trunk. Forty-three months after diagnosis, the child was still in remission.15 However, this is the only case report available noting successful treatment without surgical resection, so the effect of this chemotherapy/radiotherapy approach remains uncertain and thus cannot be recommended for MTT treatment. [...]chemo- and radiotherapies were followed by stem cell transplantation, and the contribution of stem cell transplantation to the outcome is questionable. If complete resection of the tumor is considered feasible, patients with MTT often undergo surgery in combination with adjuvant chemotherapy and/or postoperative radiation therapy.8,10 Postoperative chemotherapy and radiation therapy to the primary site can improve the chances of survival.7,9,10 In 2020, Ailing Zhao, MD, and colleagues compared the survival rates of children treated by different modalities and demonstrated that the rates were significantly improved for children who received postoperative therapies, vs those who had surgery alone.7 However, enlarged tumors and metastatic lesions limit the efficacy of surgery followed by adjuvant therapy. According to the data available in the literature, for children who received combination treatment of surgery, chemotherapy, and r