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胸壁症状性间叶性错构瘤的治疗:仅对有症状的病例进行手术切除。

Management of symptomatic mesenchymal hamartoma of the chest wall: surgical resection only in symptomatic cases.

作者信息

Virgone C, Dall'Igna P, Alaggio R, Burnelli R, Zanon G F, Cecchetto G

机构信息

Pediatric Surgery, Department of Women's and Children's Health, University-Hospital of Padua, Italy.

Pathology Unit, Department of Medical and Diagnostic Sciences and Special Therapies, University of Padua, Italy.

出版信息

Klin Padiatr. 2013 Dec;225(7):420-2. doi: 10.1055/s-0033-1354355. Epub 2013 Sep 10.

Abstract

Mesenchymal hamartoma of the chest wall (MHCW) is a rare neonatal benign tumor with an estimated incidence of 1 in 3 000 among primary bone tumors, and 1 in one million in the general population. Traditionally, the treatment of choice was an "en bloc" resection, but surgery limited to symptomatic cases, is now suggested by most authors due to the numerous cases of spontaneous regressions. We report 2 patients of symptomatic MHCW, characterized by progressive respiratory distress, who underwent surgical treatment with prompt resolution of symptoms. Surgeons and neonatologists should be aware of this rare condition and its possible fatal or nearly-fatal complications.

摘要

胸壁间叶性错构瘤(MHCW)是一种罕见的新生儿良性肿瘤,在原发性骨肿瘤中估计发病率为3000分之一,在普通人群中为百万分之一。传统上,首选的治疗方法是“整块”切除,但由于有许多自发消退的病例,现在大多数作者建议仅对有症状的病例进行手术。我们报告了2例有症状的MHCW患者,其特征为进行性呼吸窘迫,他们接受了手术治疗,症状迅速缓解。外科医生和新生儿科医生应了解这种罕见疾病及其可能的致命或近乎致命的并发症。

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