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6 岁女孩面部炎症性肌纤维母细胞瘤:罕见病例带来的整形外科学教训。

A facial inflammatory myofibroblastic tumour in a 6-year-old girl: plastic surgery lessons from a rare case.

机构信息

Plastic, Reconstructive and Aesthetic Surgery Unit, Geneva University Hospitals and Faculty of Medicine, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva 14, Switzerland.

出版信息

J Craniomaxillofac Surg. 2011 Mar;39(2):141-4. doi: 10.1016/j.jcms.2010.11.015. Epub 2010 Dec 30.

Abstract

Inflammatory myofibroblastic tumours (IMT) are rare, challenging lesions with respect to differential diagnosis, biological behaviour and treatment. We reviewed the literature and report a unique case of a large (9 × 8 × 8 cm) IMT in the nasal region of a 6-year-old girl responsible for important facial deformation. Following surgical resection, without any craniofacial reconstruction, the dysmorphism regressed spontaneously with a good result at 2 years of follow-up. To date, this is the largest IMT reported. Surprisingly, after simple excision of the tumour the craniofacial bones and soft tissues regained spontaneously their normal anatomical position only after a few months. The bone plasticity was likely due to the young age of patient and the preservation of periosteum and muscles during the surgical excision. We conclude that a conservative approach may be considered as alternative to reconstructive surgery in particular cases in young subjects.

摘要

炎性肌纤维母细胞瘤(IMT)是一种罕见的、具有挑战性的病变,在鉴别诊断、生物学行为和治疗方面存在困难。我们回顾了相关文献,并报告了一例 6 岁女孩鼻腔内罕见的大型(9×8×8cm)IMT 病例,该病例导致了严重的面部畸形。在接受了单纯的手术切除后,未进行任何颅面重建,该畸形在 2 年的随访中自发消退,取得了良好的效果。迄今为止,这是报道的最大的 IMT 病例。令人惊讶的是,在肿瘤单纯切除后,颅面骨和软组织在几个月后仅自行恢复到正常的解剖位置。骨的可塑性可能与患者年龄较小以及在手术切除过程中保留了骨膜和肌肉有关。我们的结论是,在某些特定情况下,特别是在年轻患者中,保守治疗可能是重建手术的替代方案。

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