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儿童炎性肌纤维母细胞瘤的临床影像学及病理特征:一项回顾性研究

Clinico-radiological and Pathological Characteristics of Inflammatory Myofibroblastic Tumors in Children: A Retrospective Study.

作者信息

Mir Mohamad Hussain, Dar Waseem, Aejaz Aziz Sheikh, Mohamad Gul, Wani Burhan

机构信息

Department of Medical Oncology, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.

出版信息

Indian J Med Paediatr Oncol. 2017 Jul-Sep;38(3):261-265. doi: 10.4103/ijmpo.ijmpo_178_16.

Abstract

PURPOSE

Inflammatory myofibroblastic tumors (IMTs) are rare, benign lesions most often seen in the lung of young adults but can occur in children, in various sites. They mimic, clinically and radiologically, malignant tumors - especially sarcomas and lymphomas. The aim was to review the clinical, radiological, and pathological data of children with a diagnosis of IMT referred to our department.

MATERIALS AND METHODS

This retrospective study was conducted at the Department of Medical and Paediatric Oncology, Regional Cancer Centre, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India from January 2014 to December 2015.

RESULTS

Among 288 pediatric tumors registered during the study, 5 (1.73%) had the diagnosis of IMTs. The main symptoms were abdominal distension and pain in 60% (three cases), breathlessness and cough in 20% (one case), and right axillary area swelling in 20% (one case). In three patients, complete surgical excision was done, whereas another patient with retroperitoneal mass had the residual disease and received chemotherapy followed by complete second surgery. In the case of mediastinal IMT, surgery was followed by local radiotherapy. At present, four patients are disease-free and one patient with mediastinal IMT has the residual progressive disease.

CONCLUSION

On presentation, IMT can constitute a formidable challenge, from diagnosis through to treatment.

摘要

目的

炎性肌纤维母细胞瘤(IMT)是一种罕见的良性病变,最常见于年轻成人的肺部,但也可发生于儿童的各种部位。它们在临床和放射学上与恶性肿瘤相似,尤其是肉瘤和淋巴瘤。本研究旨在回顾我院确诊为IMT的儿童患者的临床、放射学和病理学资料。

材料与方法

本回顾性研究于2014年1月至2015年12月在印度查谟和克什米尔邦斯利那加市谢里-克什米尔医学科学研究所区域癌症中心医学与儿科肿瘤学系进行。

结果

在本研究登记的288例儿童肿瘤中,5例(1.73%)被诊断为IMT。主要症状为腹胀和腹痛,占60%(3例);呼吸急促和咳嗽,占20%(1例);右腋窝区肿胀,占20%(1例)。3例患者接受了完整的手术切除,而另1例患有腹膜后肿块的患者有残留病灶,接受了化疗,随后进行了二次完整手术。对于纵隔IMT患者,手术后进行了局部放疗。目前,4例患者无疾病,1例纵隔IMT患者有残留进展性疾病。

结论

IMT从诊断到治疗都可能构成巨大挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e1b/5686963/abfb28c2c8b9/IJMPO-38-261-g001.jpg

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