Alveolar rhabdomyosarcoma of the anterior mediastinum with vessel invasion in a 4-month-old boy: a case report

Journal of Medical Case Reports

Calvin S.H. Ng¹  Malcolm J. Underwood¹  Song Wan¹  Ka Fai To²  Innes Y.P. Wan¹  Randolph H.L. Wong¹  Simon C.Y. Chow¹ 

[1] Division of Cardiothoracic Surgery, Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, New Territories Hong Kong;Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, New Territories Hong Kong

关键词: Sternotomy;    Pediatric;    Mediastinal tumor;    Rhabdomyosarcoma;    Mediastinum;   

Others  :  1231413
DOI  :  10.1186/s13256-015-0642-4

 received in 2015-01-16, accepted in 2015-06-19,  发布年份 2015

【 摘 要 】

Introduction

Alveolar rhabdomyosarcomas of the mediastinum in children are rarely reported. Multimodality therapy including chemotherapy, surgery and radiotherapy make up the backbone of the treatment of childhood rhabdomyosarcomas. Complete resection whenever achievable is an important prognostic factor. However, complete resection of tumors in the mediastinum often poses a unique challenge to thoracic surgeons due to their close proximity to important neurovascular structures. Complete resection may not always be possible and judicious peri-operative planning and preparation are required to avoid creating unnecessary surgical morbidities resulting in delay of adjuvant therapy.

Case presentation

A 4-month-old Chinese baby boy was presented to our hospital with stridor, shortness of breath and episodes of cyanosis. Imaging studies found an anterior mediastinal mass compressing the trachea and other neurovascular structures and he was diagnosed to have alveolar rhabdomyosarcoma. Our patient received upfront chemotherapy and subsequently open resection of the mass was attempted via median sternotomy. Intraoperatively, the mass had invaded into the great vessels, precluding a complete resection. Debulking surgery was performed instead and our patient received timely postoperative chemoradiotherapy.

Conclusions

We report a rare case of childhood alveolar rhabdomyosarcoma of the mediastinum with vascular invasion treated with chemoradiotherapy and debulking surgery. Complete resection was not possible due to the close proximity to the great vessels. Different surgical approaches to the mediastinum have been reported in adults and children alike. Regardless of the surgical access, the treatment of childhood rhabdomyosarcomas should be individualized, with careful balance between surgical clearance and surgical morbidity.

【 授权许可】

   
2015 Chow et al.

【 图 表 】

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