Guilmette Julie, Laverdière Caroline, Soulières Denis, Patey Natasha, Soucy Geneviève, Trudel Dominique, Bouron-Dal Soglio Dorothée
1 Department of Anatomo-Pathology, University of Montreal Heath Care Center, Montreal, Quebec, Canada.
2 Hematology-Oncology Division, Department of Pediatrics, Ste-Justine Hospital, Montreal, Quebec, Canada.
Pediatr Dev Pathol. 2017 Jun;20(3):262-266. doi: 10.1177/1093526617706814. Epub 2017 Apr 21.
Introduction Malignant rhabdoid tumor (MRT) is defined as a high-grade sarcoma derived from an uncertain cell of origin. Its diagnosis is associated with poor prognosis and patient's life expectancy is greatly reduced. Material and method Here, we describe a unique case of 9-month-old boy who presented with a large MRT arising from the soft tissue of the neck. Following intensive multimodal treatment, the patient benefited from a 25 years' remission until the discovery of multiple liver metastases. Conclusion MRT of soft tissue needs to be distinguished from other soft tissue neoplasms, as MRT is highly aggressive and is usually associated with a poor outcome. In addition, this is the longest remission time reported in a patient with soft tissue MRT and this might be related to the use of early intensive multimodal treatments.
引言
恶性横纹肌样瘤（MRT）被定义为一种起源不明细胞的高级别肉瘤。其诊断与预后不良相关，患者预期寿命大幅缩短。
材料与方法
在此，我们描述了一例独特病例，一名9个月大的男孩，颈部软组织出现巨大MRT。经过强化多模式治疗，患者缓解了25年，直至发现多处肝转移。
结论
软组织MRT需要与其他软组织肿瘤相鉴别，因为MRT具有高度侵袭性，通常预后不良。此外，这是软组织MRT患者报告的最长缓解时间，这可能与早期强化多模式治疗的使用有关。
