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下颌婴儿型硬纤维瘤病的治疗:一例儿科病例报告。

Management of mandibular infantile desmoid fibromatosis: A pediatric case report.

作者信息

Kharrat Rania, Chaabouni Mohamed Amine, Sghaier Yasmine, Akrout Ines, Hammami Bouthaina, Charfeddine Ilhem

机构信息

ENT department and research Laboratory LR23ES01, Habib Bourguiba University Hospital, University of Sfax, Sfax, Tunisia.

出版信息

Int J Surg Case Rep. 2025 Aug;133:111666. doi: 10.1016/j.ijscr.2025.111666. Epub 2025 Jul 11.

Abstract

INTRODUCTION

Infantile desmoid fibromatosis (IDF) is a rare, benign soft tissue tumor, with locally infiltrative behavior. It presents two forms: solitary and disseminated. This condition can affect any location, although IDF of the mandibular is exceptionally rare. Its locally aggressive nature and proximity to critical anatomical structures present significant management challenges. Its management modalities are still controverted.

CASE PRESENTATION

We report a rare case mandibular IDF, highlighting its clinical features and management. A two-year-old child with no known medical history, presented with a 5-month history of a right lateral cervical mass. Physical examination revealed a fixed 3 cm mass in the sub-mandibular region. Cervical computed tomography showed a 4 cm heterogeneous lesion at the right mandibular angle, with bone lysis and contact with the ipsilateral submandibular gland and the hyoid bone. A biopsy was performed and histological examination confirmed the diagnosis of an IDF. The patient underwent a wait and see approach, and after two years of follow-up, both clinical and ultrasound evaluation showed a significant regression of the mass, with no associated symptoms. No artificial intelligence (AI) tools were used in the research, analysis, or writing of this manuscript.

CLINICAL DISCUSSION

The diagnosis of IDF is challenging and relies on histological examination. The treatment is multidisciplinary and still controverted. It involves a wait and see approach, chemotherapy and surgery. Given the possibility of spontaneous regression of the lesion, a conservative approach is preferred in pediatric patients.

CONCLUSION

This study highlights a rare occurrence of a mandibular IDF in a child to emphasize the importance of making the appropriate diagnosis and management modality.

摘要

引言

婴儿型硬纤维瘤病(IDF)是一种罕见的良性软组织肿瘤,具有局部浸润性。它有两种表现形式:孤立性和播散性。这种疾病可发生于任何部位,不过下颌骨的IDF极为罕见。其局部侵袭性以及与关键解剖结构的毗邻关系给治疗带来了重大挑战。其治疗方式仍存在争议。

病例报告

我们报告一例罕见的下颌骨IDF病例,突出其临床特征及治疗情况。一名无已知病史的两岁儿童,右侧颈部肿块已有5个月病史。体格检查发现下颌下区域有一个3厘米大小的固定肿块。颈部计算机断层扫描显示右下颌角有一个4厘米大小的不均匀病变,伴有骨质溶解,并与同侧下颌下腺及舌骨接触。进行了活检,组织学检查确诊为IDF。患者采取了观察等待的方法,经过两年随访,临床及超声评估显示肿块显著缩小,且无相关症状。本手稿的研究、分析及撰写过程未使用人工智能(AI)工具。

临床讨论

IDF的诊断具有挑战性,依赖于组织学检查。治疗是多学科的,且仍存在争议。治疗方法包括观察等待、化疗和手术。鉴于病变有自发消退的可能性,小儿患者首选保守治疗方法。

结论

本研究突出了儿童下颌骨IDF的罕见病例,以强调做出恰当诊断及选择治疗方式的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9b/12275847/8afca34f71d3/gr1.jpg

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