Zheng Nannan,Peng Xuehua,Gan Tongjia,et al.Imaging characteristics and diagnostic analysis of pediatric abdominal inflammatory myofibroblastic tumors[J].Journal of Clinical Pediatric Surgery,2025,(06):570-576.[doi:10.3760/cma.j.cn101785-202504010-012]
儿童腹部炎性肌纤维母细胞瘤的影像学特征及诊疗分析
- Title:
- Imaging characteristics and diagnostic analysis of pediatric abdominal inflammatory myofibroblastic tumors
- Keywords:
- Neoplasms; Muscle Tissue; Inflammatory Myofibroblastic Tumor; Abdomen; Imaging; Three-Dimensional; Surgical Procedures; Operative; Child
- 摘要:
- 目的 探究儿童炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的临床与影像学特征,并分析多层螺旋CT(multi-slice spiral CT,MSCT)在腹部IMT诊断中的应用价值。方法 对华中科技大学同济医学院附属武汉儿童医院2010年8月至2023年9月期间,经手术病理证实的13例儿童腹部IMT患儿临床资料进行回顾性分析。患儿年龄2个月至6岁,均行MSCT平扫及增强检查,检查设备为SOMATOM公司的CT机,管电压设定为80~100 kV,并采用自动管电流调节技术以确保扫描质量。CT机的旋转速率设置为0.8 s/360°,螺距为0.984,扫描范围自膈顶至耻骨联合上缘。采用碘海醇注射液(浓度为350 mg/mL)作为对比剂,原始图像以0.6 mm层厚进行重建,并利用多平面重组(multi-planar reformation,MPR)、最大密度投影(maximum intensity projection,MIP)等技术对图像进行后处理分析。结果 13例患儿中,12例根治性手术切除成功,1例进行了组织病理活检以明确诊断。肿瘤发病部位:胃肠道8例,系膜/大网膜6例(其中1例与胃肠道重叠),膀胱1例,肝脏2例(其中1例与其他部位重叠)。单发10例,多发3例。术前实验室检查显示,白细胞升高5例,C反应蛋白(C-reactive protein,CRP)升高9例。血红蛋白(hemoglobin,HGB)降低8例,13例血小板(platelet,PLT)呈升高趋势。MSCT平扫结果显示肿瘤形态多样,包括分叶状5例、类圆形7例、斑片状1例。边界清楚10例,边界不清3例。病灶内可见钙化3例。密度均匀5例,密度不均8例。9例增强扫描显示渐进性强化特征。延迟期均匀强化4例,不均匀强化9例。按强化程度划分,明显强化11例,轻度强化2例。术前正确诊断6例,误诊或诊断不明确7例。结论 儿童腹部IMT具有多部位发病的特点,MSCT表现呈现多样化,采取临床特征结合影像学特点的诊断模式,有助于提高临床对该病的认识及诊断准确性。
- Abstract:
- Objective To investigate the clinicopathological characteristics of pediatric inflammatory myofibroblastic tumors (IMTs) and analyze the clinical value and diagnostic accuracy of multi-slice spiral CT (MSCT) in the diagnosis of abdominal IMTs. Methods This retrospective study analyzed the clinical data of 13 pediatric patients diagnosed with abdominal IMT by surgical pathology between August 2010 and September 2023.The patients were aged between 2 months and 6 years.All underwent MSCT plain and contrast-enhanced scanning using a SOMATOM CT scanner (tube voltage:80-100 kV,with automatic current modulation).The rotation speed was set at 0.8 s/360°,pitch at 0.984,and scan range from the diaphragm dome to the upper pubic symphysis.Iohexol (350 mg/mL) was used as the contrast agent.Original images were reconstructed at 0.6 mm thickness and processed using multi-planar reformation (MPR) and maximum intensity projection (MIP). Results Of the 13 children,12 underwent successful radical tumor resection,and 1 underwent biopsy for diagnosis.Tumor locations included the gastrointestinal tract (n=8),mesentery/greater omentum (n=6,with partial overlap),bladder (n=1),and liver (n=2,1 overlapping with other sites).Solitary lesions were observed in 10 cases,and multiple lesions in 3 cases.Laboratory results showed elevated white blood cell count in 5 cases,elevated C-reactive protein (CRP) in 9,decreased hemoglobin (HGB) in 8,and increased platelet count (PLT) in all cases.MSCT revealed diverse tumor morphologies:lobulated (n=5),quasi-round (n=7),and patchy (n=1).Well-defined margins were observed in 10 cases and ill-defined in 3.Intratumoral calcifications appeared in 3 cases.Tumor density was homogeneous in 5 and heterogeneous in 8.Among 9 cases undergoing contrast-enhanced scanning progressive enhancement was observed; 4 showed homogeneous and 9 showed heterogeneous delayed-phase enhancement.Based on enhancement degree,11 showed marked enhancement and 2 mild enhancement.Preoperative diagnosis was correct in 6 cases,while 7 were misdiagnosed or had inconclusive diagnosis. Conclusions Pediatric abdominal IMTs often involve multiple sites and display varied MSCT features.Integrating clinical and imaging characteristics can improve disease.
参考文献/References:
[1] 乔一丹,陶菁,何乐建.儿童上皮样炎性肌纤维母细胞肉瘤7例临床病理和分子遗传学特征[J].临床与实验病理学杂志,2024,40(7):710-713.DOI:10.13315/j.cnki.cjcep.2024.07.009. Qiao YD,Tao J,He LJ.Clinicopathological and molecular genetic characteristics of 7 cases of epithelioid inflammatory myofibroblastic sarcoma in children[J].Chin J Clin Exp Pathol,2024,40(7):710-713.DOI:10.13315/j.cnki.cjcep.2024.07.009.
[2] Pio LC,Varela P,Eliott MJ,et al.Pediatric airway tumors:a report from the International Network of Pediatric Airway Teams (INPAT)[J].Laryngoscope,2020,130(4):E243-E251.DOI:10.1002/lary.28062.
[3] Rodrigues L,Domingues R,Amaral D,et al.Primary pulmonary tumor in an adolescent[J].Acta Med Port,2020,33(7/8):512-515.DOI:10.20344/amp.12690.
[4] Dermarkarian CR,Patel KR,Fuller MY,et al.Inflammatory myofibroblastic tumor of the orbit in an 8-month old[J].Ophthalmic Plast Reconstr Surg,2020,36(3):e65-e68.DOI:10.1097/IOP.0000000000001575.
[5] Gerstle K,Siddiqui A,Schulte JJ,et al.Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient[J].Pediatr Blood Cancer,2020,67(8):e28218.DOI:10.1002/pbc.28218.
[6] Kaneva K,Wadhwani N,DiPatri AJ Jr,et al.Benign skull and subdural lesions in patients with prior medulloblastoma therapy[J].Childs Nerv Syst,2021,37(2):359-366.DOI:10.1007/s00381-020-04874-2.
[7] Thirunavukkarasu B,Roy PS,Gupta K,et al.Infantile inflammatory myofibroblastic tumor of spleen[J].Fetal Pediatr Pathol,2022,41(3):475-479.DOI:10.1080/15513815.2020.1836098.
[8] Styczewska M,Patel A,Jaskulowska J,et al.Therapeutic options in inoperable ROS1-rearranged inflammatory myofibroblastic tumor of the tongue in a child:a case report and literature review[J].Anticancer Drugs,2021,32(10):1111-1115.DOI:10.1097/CAD.0000000000001099.
[9] Wachter F,Al-Ibraheemi A,Trissal MC,et al.Molecular characterization of inflammatory tumors facilitates initiation of effective therapy[J].Pediatrics,2021,148(6):e2021050990.DOI:10.1542/peds.2021-050990.
[10] Zhou X,Song LC,Cheng YP,et al.Unusual case of a right atrial inflammatory myofibroblastic tumor presenting with an episodic cyanosis in an infant[J].J Card Surg,2022,37(9):2859-2861.DOI:10.1111/jocs.16698.
[11] Fang YW,Song HC,Sun N,et al.Non-Wilms’ renal tumors in children:experience with 139 cases treated at a single center[J].BMC Urol,2022,22(1):89.DOI:10.1186/s12894-022-01042-3.
[12] Wachter F,Janeway KA.Comment on:clinical,pathologic,and molecular features of inflammatory myofibroblastic tumors in children and adolescents:ROS1-fusion inflammatory myofibroblastic tumor:ROS1-fusion inflammatory myofibroblastic tumor[J].Pediatr Blood Cancer,2023,70(2):e29907.DOI:10.1002/pbc.29907.
[13] Song HH,Zhang HY,Zhang YF,et al.Inflammatory myofibroblastic tumor from the greater omentum in children:a rare case report[J].J Cancer Res Ther,2022,18(7):2066-2069.DOI:10.4103/jcrt.jcrt_1089_22.
[14] Tenaillon A,Orbach D.Drug approval:crizotinib in unresectable or refractory ALK-positive pediatric inflammatory myofibroblastic tumors[J].Bull Cancer,2023,110(3):248-249.DOI:10.1016/j.bulcan.2022.12.009.
[15] Zhang YL,Mo ZQ,Yang C,et al.Non-intubated tracheal resection and reconstruction for a tracheal tumor in an 8-year-old child[J].J Cardiothorac Surg,2024,19(1):468.DOI:10.1186/s13019-024-02949-8.
[16] Clore LN,Margulies IG,Baker SB.Locally aggressive and recurrent facial inflammatory myofibroblastic tumor in a pediatric patient[J].J Craniofac Surg,2025,36(1):e70-e72.DOI:10.1097/SCS.0000000000010771.
[17] ?zcan HN,Atak F,Oguz B,et al.Imaging findings of primary lung tumors in children[J].Diagn Interv Radiol,2024,30(6):419-426.DOI:10.4274/dir.2024.242714.
[18] Raitio A,Losty PD.Treatment and outcomes in pediatric inflammatory myofibroblastic tumors-a systematic review of published studies[J].Eur J Surg Oncol,2024,50(7):108388.DOI:10.1016/j.ejso.2024.108388.
[19] Reyes-Angel J,Rapkin LB,Simons JP,et al.Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child[J].Pediatr Pulmonol,2022,57(1):330-332.DOI:10.1002/ppul.25742.
[20] Mahajan P,Casanova M,Ferrari A,et al.Inflammatory myofibroblastic tumor:molecular landscape,targeted therapeutics,and remaining challenges[J].Curr Probl Cancer,2021,45(4):100768.DOI:10.1016/j.currproblcancer.2021.100768.
[21] Mittal A,Gupta A,Dhamija E,et al.Sustained complete response on crizotinib in primary lung inflammatory myofibroblastic tumor-case report and literature review[J].Monaldi Arch Chest Dis,2021,91(3).DOI:10.4081/monaldi.2021.1586.
[22] Lee EY,Vargas SO,Park HJ,et al.Thoracic multidetector computed tomography evaluation of inflammatory myofibroblastic tumor of the lung in pediatric patients in the era of modern diagnosis[J].J Thorac Imaging,2021,36(5):310-317.DOI:10.1097/RTI.0000000000000589.
[23] Chanthong S,Sathitsamitphong L,Natesirinilkul R,et al.Treatment modalities of ALK-positive relapsed/refractory inflammatory myofibroblastic tumor of the brain and lungs in 7-year-old girl:case-based reviews[J].Childs Nerv Syst,2023,39(2):331-342.DOI:10.1007/s00381-022-05789-w.
[24] 王一真,任素珍.儿童炎性肌纤维母细胞瘤11例临床病理分析[J].诊断病理学杂志,2012,19(5):355-357.DOI:10.3969/j.issn.1007-8096.2012.05.009. Wang YZ,Ren SZ.Inflammatory myofibroblastic tumor in children:a clinicopathologic study of 11 cases[J].Chin J Diagn Pathol,2012,19(5):355-357.DOI:10.3969/j.issn.1007-8096.2012.05.009.
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备注/Memo
收稿日期:2025-3-25。
通讯作者:闫学强,Email:yanxueqiang1@163.com