Jariod-Ferrer Ursula M, Trigo-Cebrian Miguel Angel, Pantilie Bianca, Simon Sanz María V, Esparza Lasaga Leire, Gavin-Clavero Marina A, Delso-Gil Elena, Martinez-Trufero Javier
Oral and Maxillofacial Department, Miguel Servet University Hospital, Paseo Isabel La Católica, nº 1-3, 50009 Zaragoza, Spain.
Pathological Anathomy Department, Miguel Servet University Hospital, Paseo Isabel La Católica nº 1-3, Zaragoza, Spain.
J Maxillofac Oral Surg. 2021 Sep;20(3):464-469. doi: 10.1007/s12663-020-01447-3. Epub 2020 Sep 26.
Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm with its origin in the skeletal muscle and is extremely rare in adults. By the World Health Organization (WHO), a new variant of RMS has been classified, i.e. the spindle cell (Sc) and sclerosing (S) RMS. While the Sc-RMS shows intersecting fascicles of nonpleomorphic spindle cells, the S-RMS is characterized by a marked hyalinization in a pseudovascular growth pattern associated with round-to-spindled tumour cells. According to the analysed data, the Sc/S-RMS variant has a worse outcome than other variants. The new classification of the Sc/S-RMS variant is valuable to the clinical practice. There are not many oral Sc/S-RMS cases reported. The aim of this paper is to demonstrate that an early diagnosis, an adequate treatment and a multidisciplinary approach have a positive effect on the prognosis of the patient. In this study, we analyse a new case of Sc-RMS variant in a young adult with an early diagnosis and a favourable outcome as a result of an appropriated multidisciplinary treatment: early surgery, radiotherapy and chemotherapy treatment.
横纹肌肉瘤(RMS)是一种起源于骨骼肌的恶性软组织肿瘤,在成人中极为罕见。世界卫生组织(WHO)已将RMS的一种新变体进行了分类,即梭形细胞(Sc)和硬化性(S)RMS。Sc-RMS表现为非多形性梭形细胞的交叉束状排列,而S-RMS的特征是在与圆形至梭形肿瘤细胞相关的假血管生长模式中出现明显的玻璃样变。根据分析数据,Sc/S-RMS变体的预后比其他变体更差。Sc/S-RMS变体的新分类对临床实践具有重要价值。报道的口腔Sc/S-RMS病例并不多。本文的目的是证明早期诊断、适当治疗和多学科方法对患者的预后有积极影响。在本研究中,我们分析了一名年轻成人Sc-RMS变体的新病例,该病例早期诊断,并因采用了适当的多学科治疗(早期手术、放疗和化疗)而获得了良好的预后。