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横纹肌肉瘤患儿和青少年的乳房转移:意大利软组织肉瘤委员会的经验。

Breast metastases in children and adolescents with rhabdomyosarcoma: Experience of the Italian Soft Tissue Sarcoma Committee.

机构信息

Pediatric Hematology and Oncology Unit, G. Di Cristina Children's Hospital, Palermo, Italy.

出版信息

Pediatr Blood Cancer. 2010 Dec 15;55(7):1306-9. doi: 10.1002/pbc.22729. Epub 2010 Aug 20.

Abstract

BACKGROUND

Breast metastasis from rhabdomyosarcoma (RMS) is an uncommon event but may be problematic in treatment decision-making. Aim of the study was to evaluate clinical characteristics, treatment, and subsequent outcome, of patients with RMS metastasis in the breast, enrolled in four consecutive Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) Soft Tissue Sarcoma Committee protocols during the last 20 years, in order to obtain information to establish a more adequate diagnostic and therapeutic approach.

PROCEDURES

Data were derived from the AIEOP STSC database and reviewed for the purpose of this study.

RESULTS

From 1988 to 2008, among 189 patients with metastatic RMS, we identified 7 (3.7%) patients with RMS with breast involvement at diagnosis. All patients were females, aged 13-17 years with alveolar histology and multiple metastasis sites (2-5). The primary tumor was located in the extremities in 3/7 patients. In spite of intensive treatment no patient survived. The cause of treatment failure was distant relapse in six patients, including two on the mammary region. Treatment data analysis revealed that local measures to control breast lesions were used in only two patients.

CONCLUSIONS

Our data suggest that investigations of the mammary region should be part of the usual diagnostic workup in adolescent girls with alveolar RMS, especially if the primary tumor arises in the extremities. New and more effective strategies are needed to improve the outcome of these patients including aggressive local measures to control breast disease.

摘要

背景

横纹肌肉瘤(RMS)的乳房转移较为少见,但可能会对治疗决策造成影响。本研究旨在评估过去 20 年中,4 个连续的意大利儿科血液学和肿瘤学会(AIEOP)软组织肉瘤委员会方案中,患有乳房 RMS 转移的患者的临床特征、治疗和后续结果,以获取相关信息来建立更合适的诊断和治疗方法。

方法

本研究从 AIEOP STSC 数据库中提取数据进行分析。

结果

1988 年至 2008 年期间,在 189 例转移性 RMS 患者中,我们发现有 7 例(3.7%)患者在诊断时存在乳房 RMS 转移。所有患者均为女性,年龄为 13-17 岁,组织学类型为肺泡型,且存在多个转移部位(2-5 个)。3/7 例患者的原发肿瘤位于四肢。尽管进行了强化治疗,但没有患者存活。6 例患者(包括 2 例乳房区域)因远处复发而导致治疗失败。治疗数据分析显示,仅 2 例患者采用局部措施来控制乳房病变。

结论

我们的数据表明,对于患有肺泡 RMS 的青春期少女,尤其是当原发肿瘤位于四肢时,应将乳房区域的检查纳入常规诊断程序中。需要采取新的、更有效的策略来改善这些患者的预后,包括积极的局部措施来控制乳房疾病。

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