Bian Yanyan, Yongbo Xiang, Xi Zhou, Zhao Dachun, Wu Huanwen, Liu Yong
Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College.
Peking Union Medical College, Clinical Medicine School.
Medicine (Baltimore). 2019 Sep;98(36):e16797. doi: 10.1097/MD.0000000000016797.
Malignant triton tumor (MTT) is an extraordinarily uncommon and aggressive tumor which have poor prognosis. Malignant peripheral nerve sheath tumors with additional rhabdomyoblasts are found in MTT histologically. The prognosis of patients is poor. The goal of our study is to describe the largest number of cases characteristic and outcome, to our knowledge, such a presentation was not described in the English-language literature until now.From 1999 to 2014, 10 patients (5 women and 5 men) with a malignant triton tumor were treated at our institution. All these cases were followed-up and patient charts were analyzed for outcome.In our study, 3 cases of the Malignant triton tumors originate in the head, 2 cases in the joints, 2 cases in the retroperitoneum, 2 cases in the soft tissues of the thoracic wall, and 1 case in the prostate. Neoplasm associated with pain was the main manifestation. Patients have a poor prognosis. Completely surgical excision of the tumor is the only treatment. Additional radiation or chemotherapy show little effect.Malignant triton tumor is a rare sarcoma. The high probability of developing local recurrence and distant metastases could account for its poor prognosis.
恶性蝾螈瘤（MTT）是一种极其罕见且侵袭性强的肿瘤，预后较差。恶性蝾螈瘤在组织学上表现为伴有横纹肌母细胞的恶性外周神经鞘瘤。患者预后不佳。据我们所知，本研究的目的是描述数量最多的病例特征及预后情况，此前英文文献中尚未有此类报道。1999年至2014年，我院共收治10例恶性蝾螈瘤患者（5例女性，5例男性）。对所有病例进行了随访，并分析患者病历以了解预后情况。在我们的研究中，3例恶性蝾螈瘤起源于头部，2例起源于关节，2例起源于腹膜后，2例起源于胸壁软组织，1例起源于前列腺。肿瘤相关疼痛是主要表现。患者预后较差。肿瘤完整手术切除是唯一的治疗方法。额外的放疗或化疗效果甚微。恶性蝾螈瘤是一种罕见的肉瘤。局部复发和远处转移的高发生率可能是其预后较差的原因。
