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成人横纹肌肉瘤(RMS)患者的基线特征、预后因素及治疗结果

Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS).

作者信息

Rab Saif Ur, Bin Naeem Sameen, Baloch Naqib Ullah, Jhatial Mussadique Ali, Waheed Muhammad, Fasih Samir, Kalsoom Awan Umm-E

机构信息

Medical Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, PAK.

出版信息

Cureus. 2022 Dec 26;14(12):e32961. doi: 10.7759/cureus.32961. eCollection 2022 Dec.

Abstract

BACKGROUND

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, while in adults it is one of the rarer tumors. Its prognosis is better in children with current treatment modalities; however, it carries poorer prognosis in adults. Recent data on adult RMS is scarce from our part of world. We report outcomes of adult patients with RMS, and with 40 patients; it is the first study to publish such a large data from Pakistan.

METHODS

This was a retrospective study that included 64 adult patients aged 18 years and older. After data extraction and scrutiny, a total of 40 patients were segregated with diagnosis of RMS of various varieties who were treated and followed up subsequently. International Business Machines (IBM) Statistical Package for Social Sciences (SPSS), version 26 (IBM Corp., Armonk, NY) was used to evaluate all of the gathered data.

RESULTS

Embryonal RMS (ERMS) was the most common subtype. Factors favoring better overall survival (OS) at 5 years were absence of nodal and distal metastases, treatment with surgery, margin negative resection, and absence of residual disease on postoperative imaging. Adjuvant radiation therapy (XRT) for positive resection margins as well as for residual disease on postoperative imaging also favored better OS at 5 years. Chemotherapy did impart a trend towards better OS; however, it was not significant. Histopathologic subtype and tumor size did not have any significant impact on outcomes. Median progression free survival (PFS) was 11 months and median OS was 15 months.

CONCLUSIONS

Adult RMS is a rare disease entity with widely heterogeneous clinical picture and poorer outcomes as compared to the disease of childhood and adolescence. Further prospective studies with larger sample size are required to establish role of patient, disease, and treatment-related factors affecting outcomes in our population.

摘要

背景

横纹肌肉瘤(RMS)是儿童最常见的软组织肉瘤,而在成人中则是较为罕见的肿瘤之一。采用当前的治疗方式,儿童患者的预后较好;然而,成人患者的预后较差。在我们所在的地区,关于成人RMS的最新数据稀缺。我们报告了成人RMS患者的治疗结果,本研究纳入了40例患者,这是首次发表来自巴基斯坦的如此大规模数据的研究。

方法

这是一项回顾性研究,纳入了64例18岁及以上的成年患者。在进行数据提取和审查后,共筛选出40例被诊断为不同类型RMS的患者,随后对其进行治疗和随访。使用国际商业机器公司(IBM)的社会科学统计软件包(SPSS)第26版(IBM公司,纽约州阿蒙克)对所有收集的数据进行评估。

结果

胚胎型RMS(ERMS)是最常见的亚型。有利于5年总生存期(OS)更好的因素包括无淋巴结和远处转移、手术治疗、切缘阴性切除以及术后影像学检查无残留疾病。对于切缘阳性以及术后影像学检查有残留疾病的患者,辅助放疗(XRT)也有利于5年时获得更好的OS。化疗确实有使OS改善的趋势;然而,并不显著。组织病理学亚型和肿瘤大小对治疗结果没有任何显著影响。中位无进展生存期(PFS)为11个月,中位OS为15个月。

结论

成人RMS是一种罕见的疾病实体,与儿童和青少年期疾病相比,临床表现广泛异质且预后较差。需要开展更大样本量的进一步前瞻性研究,以确定影响我国人群治疗结果的患者、疾病和治疗相关因素的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b982/9875868/08d0aa794e62/cureus-0014-00000032961-i01.jpg

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