下肢恶性“海象”肿瘤，有骨折病史。
Malignant "triton" tumor of the lower extremity with a history of fracture.
机构信息
Department of Orthopedic Surgery, Centro Hospitalar Universitário de Santo António, Porto, Portugal; Department of Anatomy, School of Medicine and Biomedical Sciences - University of Porto, Portugal.
Department of Orthopedic Surgery, Centro Hospitalar Universitário de Santo António, Porto, Portugal.
出版信息
Rev Esp Patol. 2024 Jan-Mar;57(1):59-63. doi: 10.1016/j.patol.2023.06.004. Epub 2023 Sep 9.
INTRODUCTION
Malignant triton tumor (MTT) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor consisting of a neurogenic tumor with rhabdomyoblastic differentiation. Only 170 cases have been reported to date, two-thirds occurring in young patients with neurofibromatosis type 1 and the remaining third presenting as a sporadic tumor.
CASE PRESENTATION
We present the case of a 49-year-old man with a sporadic grade 2 MTT of the lower limb which had had a previous tibial fracture. The patient underwent an above-knee amputation. Five months post-operatively metastases were present in the liver and vertebral column causing compression of the spinal cord, so decompressive radiotherapy and palliative chemotherapy were initiated.
CONCLUSION
Due to the precocious spread of the disease, we would suggest that adjuvant chemotherapy be considered for the eradication of micrometastases. To our knowledge, this is only the second reported case of an MTT arising in a site with a history of previous severe trauma.
简介
恶性蝾螈瘤（MTT）是一种罕见且侵袭性的外周神经鞘瘤亚型，由具有横纹肌样分化的神经性肿瘤组成。迄今为止，仅报告了 170 例病例，其中三分之二发生在伴有神经纤维瘤病 1 型的年轻患者中，其余三分之一为散发性肿瘤。
病例介绍
我们报告了一例 49 岁男性下肢散发性 2 级 MTT 的病例，该患者曾有胫骨骨折史。患者接受了膝关节以上截肢。术后 5 个月，肝脏和脊柱转移导致脊髓受压，因此开始进行减压放疗和姑息性化疗。
结论
由于疾病早期扩散，我们建议考虑辅助化疗以清除微转移灶。据我们所知，这是第二例既往有严重创伤史的部位发生 MTT 的报告病例。
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