非典型畸胎瘤样横纹肌样肿瘤伴瘤内进展:伴有转移级联的长期疾病(109个月)积极手术入路的意义

V. Karakhan, E. Prozorenko, G. Mentkevich, I. Dolgopolov, N. V. Sevyan, D. M. Belov
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摘要

本文报告一例非典型畸胎瘤样横纹肌样肿瘤,其病程最长(109个月),生活质量高。首先,在一名女性患者身上发现了一个巨大的额叶肿瘤,肿瘤进展到侧脑室,并被完全切除了。病理诊断为原始神经外胚层肿瘤。经放疗、高剂量多药化疗和细胞免疫治疗(包括鞘内治疗)联合治疗,4年无复发。复发的肿瘤切除。第二次缓解期为34个月。随后,观察到肿瘤通过脑和脊髓的脑脊液束多期转移。两次显微手术治疗,去除症状性转移的脑室Iv和III。经反复形态学检查,证实为非典型畸胎瘤样横纹肌样瘤。所提出的临床病例表明,积极的手术策略在有效的长期联合治疗这种严重的病理的作用。
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Atypical teratoid rhabdoid tumor with intratumoral advancement: significance of active surgical approach in long-term disease (109 months) with metastatic cascade
The article presents a clinical case of atypical teratoid rhabdoid tumor with one of the longest catamnesis described in literature (109 months) and high quality of life. first, a giant tumor of the frontal lobe with advancement into a lateral ventricle was found in a female patient and totally removed. It was histologically diagnosed as primitive neuroectodermal tumor. Combination treatment consisting of radiotherapy, high-dose polychemotherapy and cellular immunotherapy including intrathecal therapy led to 4-year recurrence-free period. Recurrence of the tumor was resected. The period of the 2nd remission was 34 months. Subsequently, multistage metastasis of the tumor through cerebrospinal fluid tracts of the brain and spinal cord was observed. Twice microsurgical treatment with removal of symptomatic metastases in ventricles Iv and III was performed. In repeat morphological examinations, the tumor was verified as atypical teratoid rhabdoid tumor. The presented clinical case shows the role of active surgical tactics in effective long-term combination treatment of this severe pathology.
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