婴儿小肠炎性肌成纤维细胞瘤1例

IF 0.2 Q4 PEDIATRICS
Moaied A. Hassan , Halah Raheem Mohammed
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引用次数: 0

摘要

炎症性肌纤维母细胞瘤是一种罕见的、实体的、中等级别的间充质肿瘤,主要发生在儿童和青少年。婴儿病例极为罕见。病例介绍:一名6个月大的女婴因腹痛、反复非胆汁性呕吐和苍白病史转至我们的机构,腹部超声显示左侧腹部有一个很大(120✕76 mm)的肿块。除反复发作腹痛外,无小肠梗阻症状。外周血涂片示正色贫血伴中度血小板增多。其他调查,包括随机血糖、肾功能和肝功能测试、血清电解质和凝血分析,均在正常范围内。腹部计算机断层扫描证实左侧肾上区有一个大的(10✕10✕9 cm)实性非均匀增强肿块,横跨中线,提示神经母细胞瘤。剖腹探查发现在回肠中部的反肠系膜边界有一个大的分叶状肿块。完全手术切除游离肠缘。组织病理检查示:肌成纤维梭形细胞增生,轻度异型性,有丝分裂活性低,伴炎性(淋巴细胞和浆细胞)浸润,细胞高、细胞低区排列,伴黏液样口,表现为炎性肌成纤维细胞瘤。免疫组化证实标本间变性淋巴瘤激酶呈阴性。手术切除后,患者未接受进一步的辅助治疗,并保持无症状,术后连续四个半月的超声检查结果正常。结论虽然罕见,但在有腹部大肿瘤和腹痛的婴儿中应考虑炎性肌成纤维细胞瘤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Infantile inflammatory myofibroblastic tumor of the small bowel: a case report

Introduction

Inflammatory myofibroblastic tumors are rare, solid, intermediate-grade mesenchymal neoplasms that predominantly affect children and adolescents. Infantile cases are exceedingly rare.

Case presentation

A 6-month-old female infant was referred to our institution with a history of abdominal pain, repeated non-bilious vomiting and pallor, with an abdominal ultrasound that revealed the presence of a large (120✕76 mm) left sided abdominal mass. Apart from recurrent attacks of vague abdominal pain, there were no features of small bowel obstruction. Peripheral blood smear revealed normochromic anemia with moderate thrombocytosis. Other investigations, including random blood sugar, renal and liver function tests, serum electrolytes, and coagulation profile, were all within normal limits. Abdominal computed tomography confirmed the presence of a large (10✕10✕9 cm), solid heterogeneously enhanced mass in the left suprarenal region, crossing the midline, with the suggestion of neuroblastoma. Exploratory laparotomy revealed a large lobulated mass at the antimesenteric border of the mid-ileum. Complete surgical resection was performed with free intestinal margins. Histopathological examination revealed myofibroblastic spindle cell proliferation with mild atypia and low mitotic activity admixed with inflammatory (lymphocyte and plasma cell) infiltration, arranged in hyper- and hypocellular area with myxoid stoma, features in favor of inflammatory myofibroblastic tumor. Immunohistochemistry confirmed the specimen was negative for anaplastic lymphoma kinase. Following surgical excision, the patient received no further adjuvant therapy and remained asymptomatic, with normal ultrasound findings on four successive bimonthly postoperative visits.

Conclusion

Although rare, inflammatory myofibroblastic tumors should be considered in infants who have large abdominal tumors and abdominal pain.
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来源期刊
CiteScore
0.60
自引率
25.00%
发文量
348
审稿时长
15 days
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