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儿童巨大肋骨尤文肉瘤的多学科整块切除及同期胸壁重建:病例报告

Multidisciplinary en-bloc resection and concurrent chest wall reconstruction for a massive rib Ewing sarcoma in a child: A case report.

作者信息

Toumi Afef, Mosbahi Sana, Belhassen Samia, Taieb Cherif, Mahdhi Nidhal, Sahnoun Lassaad

机构信息

Pediatric Surgery Department, Fattouma Bourguiba University Hospital, Monastir, Tunisia.

Pediatric Surgery Department, Fattouma Bourguiba University Hospital, Monastir, Tunisia.

出版信息

Int J Surg Case Rep. 2025 Jun;131:111404. doi: 10.1016/j.ijscr.2025.111404. Epub 2025 May 3.

DOI:10.1016/j.ijscr.2025.111404
PMID:40327913
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12127596/
Abstract

BACKGROUND

Ewing sarcoma of the rib is a rare and aggressive malignancy in children, requiring a multidisciplinary approach for optimal management. Complete tumor resection is crucial for survival but often results in extensive chest wall defects, necessitating complex reconstruction. This case highlights the successful en-bloc resection and simultaneous chest wall reconstruction in a pediatric patient, emphasizing the role of a coordinated surgical strategy.

CASE PRESENTATION

A 10-year-old girl presented with progressive chest pain and a palpable anterior thoracic mass. Imaging revealed a large Ewing sarcoma of the left 6th rib with intra- and extrathoracic extension. Following neoadjuvant chemotherapy per the EuroEwing 2012 protocol, she underwent en-bloc resection of the 5th, 6th, and 7th ribs with a wedge lung resection. Chest wall reconstruction was performed using a polypropylene mesh reinforced with a latissimus dorsi flap and an anterior serratus muscle layer. The postoperative course was uneventful, and she remained tumor-free at a two-year follow-up.

CLINICAL DISCUSSION

Managing pediatric Ewing sarcoma of the chest wall is challenging due to the need for oncologic clearance while maintaining thoracic stability and function. The choice of reconstructive technique must balance skeletal integrity, pulmonary mechanics, and future growth. In this case, the combination of synthetic mesh and autologous muscle flaps provided structural support and soft tissue coverage, enabling effective adjuvant radiotherapy. A multidisciplinary team was essential for achieving optimal oncological and functional outcomes.

CONCLUSION

En-bloc tumor resection with immediate, well-planned reconstruction can lead to favorable oncological and functional outcomes, improving survival and quality of life.

摘要

背景

肋骨尤因肉瘤是儿童中一种罕见且侵袭性强的恶性肿瘤,需要多学科方法进行最佳管理。完整的肿瘤切除对生存至关重要,但往往会导致广泛的胸壁缺损,需要进行复杂的重建。本病例突出了在一名儿科患者中成功进行整块切除并同时进行胸壁重建,强调了协调手术策略的作用。

病例介绍

一名10岁女孩出现进行性胸痛和可触及的前胸肿物。影像学检查显示左第6肋骨有一个巨大的尤因肉瘤,累及胸腔内和胸腔外。按照2012年欧洲尤因肉瘤方案进行新辅助化疗后,她接受了第5、6和7肋骨的整块切除及楔形肺切除术。使用聚丙烯网片并辅以背阔肌皮瓣和前锯肌层进行胸壁重建。术后过程顺利,在两年随访时她仍无肿瘤。

临床讨论

由于需要在保持胸部稳定性和功能的同时进行肿瘤清除,管理儿童胸壁尤因肉瘤具有挑战性。重建技术的选择必须在骨骼完整性、肺力学和未来生长之间取得平衡。在本病例中,合成网片和自体肌皮瓣的组合提供了结构支撑和软组织覆盖,使有效的辅助放疗成为可能。多学科团队对于实现最佳肿瘤学和功能结果至关重要。

结论

整块肿瘤切除并立即进行精心规划的重建可带来良好的肿瘤学和功能结果,提高生存率和生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8f7/12127596/da2167e59e13/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8f7/12127596/d5766c3d2ef2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8f7/12127596/da2167e59e13/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8f7/12127596/d5766c3d2ef2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8f7/12127596/da2167e59e13/gr2.jpg

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