Advanced Metastatic Malignant Triton Tumor in Neurofibromatosis Type 1: A Case Report and Management Challenges.

BACKGROUND/AIM: A malignant Triton tumor (MTT) is a rare and aggressive soft tissue sarcoma associated with poor prognosis and with no established treatment protocol.

CASE REPORT: A 38-year-old man presented with severe right thigh pain and limited hip motion. Since childhood, the patient had a mass on their thigh, accompanied by café-au-lait spots. Upon admission, a biopsy confirmed MTT that had metastasized to the lungs. Preoperative radiation therapy was administered to reduce the tumor size; however, the tumor did not shrink. Extensive resection was not feasible because of the tumor size and location, prompting a decision to perform volume reduction surgery aimed at alleviating the patient's pain and improving mobility. Although the surgery provided temporary relief from the symptoms, the patient died two weeks later.

CONCLUSION

MTT requires a multidisciplinary approach that includes surgery, chemotherapy, and radiation therapy; however, in advanced cases such as this one, palliative measures may be more appropriate. This case underscores the challenges in managing MTT and highlights the potential role of volume reduction surgery in improving the quality of life of patients with significant symptoms. Despite the poor prognosis, symptom palliation during the two weeks leading up to the patient's death was significant, illustrating the importance of addressing pain and mobility issues while considering overall treatment strategies in such complex cases. These findings emphasize the need for further research on effective management options for MTT to improve patient outcomes.