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小儿炎性肌纤维母细胞瘤罕见病例的成功管理:一例报告及文献综述

Successful management of an unusual case of pediatric inflammatory myofibroblastic tumor: a case report and literature review.

作者信息

Amro Alhareth M, Almassri Tabarak, Albandak Maram, Elqadi Mohammad, Bannoura Sami, Asafrah Anas A, Abu Asbeh Yousef

机构信息

Medical Research Club, Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.

Surgery Department, Al-Ahli Hospital, Hebron, Palestine.

出版信息

Ann Med Surg (Lond). 2025 Jan 9;87(1):407-412. doi: 10.1097/MS9.0000000000002870. eCollection 2025 Jan.

DOI:10.1097/MS9.0000000000002870
PMID:40109596
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11918599/
Abstract

INTRODUCTION AND IMPORTANCE

Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic condition that primarily affects children and young adults. This case report highlights the challenges in diagnosing and treating pediatric IMT, emphasizing the importance of tailored interventions.

CASE PRESENTATION

An 8-year-old boy presented with respiratory symptoms and was diagnosed with an IMT located in the left main bronchus. Imaging studies revealed a soft tissue mass and lymph node enlargement. Bronchoscopy and biopsy confirmed the diagnosis. The patient underwent bronchoscopic debulking procedures followed by lobectomy and bronchoplasty due to persistent disease.

CLINICAL DISCUSSION

IMTs pose diagnostic challenges due to their varied clinical presentation and similarities with other neoplasms. A multidisciplinary approach involving pathologists, radiologists, and surgeons is crucial for accurate diagnosis and optimal treatment planning. The pathogenesis of IMTs is not fully understood, but theories suggest an inflammatory response or involvement of the ALK gene. IMTs can affect various organs, each with distinct symptoms. Imaging modalities lack specificity, emphasizing the importance of histopathological examination.

CONCLUSION

IMTs require individualized treatment approaches based on the location and extent of the tumor. Long-term follow-up is essential for monitoring recurrence and metastasis. Further research is needed to enhance our understanding of IMT biology and develop targeted therapies to improve patient outcomes. This case report underscores the importance of tailored interventions in pediatric IMT cases and highlights the challenges in diagnosis and treatment.

摘要

引言与重要性

炎性肌纤维母细胞瘤(IMT)是一种罕见的肿瘤性疾病,主要影响儿童和年轻人。本病例报告强调了小儿IMT诊断和治疗中的挑战,强调了量身定制干预措施的重要性。

病例介绍

一名8岁男孩出现呼吸道症状,被诊断为位于左主支气管的IMT。影像学检查显示软组织肿块和淋巴结肿大。支气管镜检查和活检确诊。由于疾病持续存在,患者接受了支气管镜减瘤手术,随后进行了肺叶切除术和支气管成形术。

临床讨论

IMT因其临床表现多样且与其他肿瘤相似而带来诊断挑战。由病理学家、放射科医生和外科医生组成的多学科方法对于准确诊断和优化治疗方案至关重要。IMT的发病机制尚未完全了解,但理论表明存在炎症反应或ALK基因的参与。IMT可影响各个器官,每个器官都有不同的症状。影像学检查缺乏特异性,强调了组织病理学检查的重要性。

结论

IMT需要根据肿瘤的位置和范围采取个体化的治疗方法。长期随访对于监测复发和转移至关重要。需要进一步研究以增强我们对IMT生物学的理解,并开发靶向治疗以改善患者预后。本病例报告强调了小儿IMT病例中量身定制干预措施的重要性,并突出了诊断和治疗中的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/45a1dbb21bf3/ms9-87-407-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/6d49f2ca31b8/ms9-87-407-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/c307511108a4/ms9-87-407-g002.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/775efbfbc216/ms9-87-407-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/45a1dbb21bf3/ms9-87-407-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/6d49f2ca31b8/ms9-87-407-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/c307511108a4/ms9-87-407-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/277916fe29dc/ms9-87-407-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20f3/11918599/563251a7ee84/ms9-87-407-g004.jpg
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