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儿童头颈部肉瘤:一项来自国家三级转诊中心的回顾性研究。

Pediatric head and neck sarcomas: a retrospective study from a national tertiary referral center.

作者信息

Bandora Eiman Abu, Kampel Liyona, Manisterski Michal, Elhasid Ronit, Levin Dror, Horowitz Gilad, Warshavsky Anton, Wolf Raphael, DeRowe Ari, Muhanna Nidal

机构信息

Department of Otolaryngology, Head and Neck and Maxillofacial Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel, Affiliated to the Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Department of Pediatric Hemato-Oncology, Dana Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel, Affiliated to the Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

Eur J Pediatr. 2025 Feb 1;184(2):169. doi: 10.1007/s00431-025-05991-3.

Abstract

UNLABELLED

This study aims to analyze the demographics, therapeutic approaches, and outcomes of pediatric sarcomas of the head and neck treated at a single tertiary referral center. We retrospectively reviewed the medical charts of all pediatric patients diagnosed with head and neck sarcomas treated at the Tel Aviv Sourasky Medical Center during 2002-2021. Clinical data, oncologic and surgical treatments, and outcome measures were retrieved from electronic medical files. A total of 52 patients met the inclusion criteria. The mean age at diagnosis was 7.25 ± 6.04 years (range 2 months to 20 years), and the male-to-female ratio was 1.4: 1. The leading histological subtypes were rhabdomyosarcoma (RMS) (50%) followed by Ewing sarcoma (23%). The vast majority (96%) of patients were treated with multimodal therapy that included chemotherapy, surgical resection, and/or radiation therapy. Twenty-nine (55.8%) patients underwent surgical resection. The average follow-up was 4.75 years (range 5 months to 13.5 years). The 1-, 5-, and 10-year overall survival rates were 89.5%, 81.3%, and 62.5%, respectively. Male sex, Ewing sarcoma, and non-metastatic disease at diagnosis were associated with better disease-free survival (DFS) (p = 0.008, p = 0.048, and p = 0.038, respectively). Patients with sarcomas suitable for surgical resection and those who received adjuvant therapy had significantly better DFS (p = 0.003 and p = 0.025, respectively).

CONCLUSIONS

Head and neck sarcomas in the pediatric population are best managed by a multidisciplinary team. Surgical resection and the integration of adjuvant therapy in selected patients confer survival benefit.

WHAT IS KNOWN

• Head and neck sarcomas pose a considerable challenge to physicians due to their rarity, their heterogenic presentation, and their proximity to vital structures.

WHAT IS NEW

• A multimodal therapeutic approach that includes chemotherapy, radiotherapy, and surgical resection when feasible should be provided to these patients in order to optimize survival outcomes. Surgical resection and the integration of adjuvant therapy in selected patients confer survival benefit.

摘要

未标注

本研究旨在分析在单一三级转诊中心接受治疗的小儿头颈部肉瘤的人口统计学特征、治疗方法及治疗结果。我们回顾性分析了2002年至2021年期间在特拉维夫索拉斯基医疗中心接受诊断和治疗的所有小儿头颈部肉瘤患者的病历。从电子病历中获取临床数据、肿瘤学及外科治疗情况以及预后指标。共有52例患者符合纳入标准。诊断时的平均年龄为7.25±6.04岁(范围为2个月至20岁),男女比例为1.4:1。主要组织学亚型为横纹肌肉瘤(RMS)(50%),其次是尤因肉瘤(23%)。绝大多数(96%)患者接受了包括化疗、手术切除和/或放疗在内的多模式治疗。29例(55.8%)患者接受了手术切除。平均随访时间为4.75年(范围为5个月至13.5年)。1年、5年和10年总生存率分别为89.5%、81.3%和62.5%。男性、尤因肉瘤以及诊断时无转移疾病与更好的无病生存率(DFS)相关(分别为p = 0.008、p = 0.048和p = 0.038)。适合手术切除的肉瘤患者以及接受辅助治疗的患者DFS明显更好(分别为p = 0.003和p = 0.025)。

结论

小儿头颈部肉瘤最好由多学科团队进行管理。手术切除以及在部分患者中整合辅助治疗可带来生存获益。

已知信息

• 头颈部肉瘤因其罕见性、异质性表现以及临近重要结构,给医生带来了相当大的挑战。

新信息

• 对于这些患者,应提供一种多模式治疗方法,包括化疗、放疗以及在可行时进行手术切除,以优化生存结果。手术切除以及在部分患者中整合辅助治疗可带来生存获益。

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