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

1型神经纤维瘤病合并晚期转移性恶性Triton肿瘤:病例报告及治疗挑战

阅读:2

Abstract

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.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。