儿童腹腔炎性肌纤维母细胞瘤的诊断学特征并文献复习

Diagnostic features of intraperitoneal inflammatory myofibroblastic tumor in children and literature review

目的:探讨儿童腹腔炎性肌纤维母细胞瘤(IMT)的诊断学特征。方法:回顾性分析山东第一医科大学附属省立医院(山东省立医院)小儿外科2021年10个月及2023年9月收治的2例腹腔IMT患儿的临床资料,结合文献总结其临床诊断学特征。结果:2例患儿均为女性,年龄分别为4岁10个月和12岁,主诉均为上腹部间断性疼痛伴发热。实验室检查:2例患儿均有不同程度血红蛋白水平降低,肿瘤标志物未见明显异常;低龄患儿C反应蛋白、降钙素原水平升高。腹腔超声提示低龄患儿为左肾上腺区低回声包块,大龄患儿为脾门处低回声包块。腹部CT平扫+增强扫描:低龄患儿提示脾胃之间腹腔内肿块,血供丰富,增强扫描不均匀强化,似与脾脏关系密切,不除外恶性;大龄患儿提示脾门处低回声肿块,可见来自脾动脉血供,增强扫描不均匀强化,考虑恶性。2例均未见周边侵犯,无腹腔积液及肿大淋巴结。2例患儿均行胸部CT、放射性核素骨扫描、骨髓穿刺检查,证实无肺、骨转移征象。低龄患儿因包块较大行开放手术腹腔病损切除术,大龄患儿行腹腔镜下腹腔病损切除术治疗,均完整切除肿瘤,术后病理诊断均为IMT。术后分别随访25个月及3个月,未见复发及转移。结论:儿童腹腔IMT无特异性临床表现及辅助检查结果,术前诊断较为困难。手术完整切除是治疗的关键,术后病理学检查可明确诊断。

Objective To explore the diagnostic characteristics of intraperitoneal inflammatory myofibroblastic tumor(IMT)in children.Methods A retrospective analysis was performed on the clinical data of 2 pediatric patients with intraperitoneal IMT who were admitted to the Department of Pediatric Surgery at Shandong Provincial Hospital Affiliated to Shandong First Medical University in October 2021 and September 2023,respectively.The clinical diagnostic characteristics were summarized using information from the literature.Results Both patients were females,aged 4 years,10 months,and 12 years,respectively.The primary complaints were intermittent upper stomach pain and fever.Laboratory studies revealed varied degrees of hemoglobin decrease in both patients,although tumor markers were not markedly abnormal.The levels of C-reactive protein and procalcitonin were increased in the younger patient.In the younger individual,abdominal ultrasound revealed a hypoechoic mass in the left adrenal area,while the elder patient showed a hypoechoic mass at the splenic hilum.Abdominal CT plain and enhanced scans revealed an intra-abdominal tumor between the spleen and stomach in the younger individual,a rich blood supply,uneven enhancement on the enhanced scan,a tight relationship to the spleen,and malignancy could not be excluded.In the elderly patient,a hypoechoic mass near the splenic hilum was discovered,supplied by the splenic artery,and an enhanced scan revealed uneven enhancement,indicating malignancy.Both patients showed no invasion of adjacent tissues,abdominal fluid buildup,or swollen lymph nodes.Both patients underwent chest CT,radionuclide bone scan,and bone marrow puncture,which revealed no evidence of lung or bone metastases.Due to the high size of the tumor,the younger patient required open surgery to completely resect the abdominal lesion,whereas the elder patient underwent laparoscopic surgery.Both procedures resulted in total tumor removal,and the postoperative pathology diagnosis confirmed IMT.Postoperative follow-up for 25-month and 3-month respectively revealed no recurrence or metastases.Conclusions IMT in the pediatric abdomen lacks distinct clinical signs and auxiliary testing procedures,making preoperative diagnosis problematic.Comprehensive surgical resection is required for treatment,and a postoperative pathology evaluation can confirm the diagnosis.