5.5 个月大男孩的原发性肝脏神经母细胞瘤:病例报告。

IF 1 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL
Farzad Kompani, Alieh Safari Sharari, Elmira Haji Esmaeil Memar, Mahya Ghahremanloo
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引用次数: 0

摘要

在儿科病例中,最常见的颅外实体瘤是神经母细胞瘤(NB),几乎总是发生在有交感神经支配的组织中,只有少数病例报道发生在其他器官。肝脏受累的神经母细胞瘤通常是转移性病变,因为原发性肝脏神经母细胞瘤极为罕见。本研究介绍了一名患有原发性肝NB的5.5个月大男孩。本病例研究描述了一名 5.5 个月大的早产男婴,他出现了明显的肝脏肿大。实验室检查显示甲胎蛋白水平异常高。在超声引导下进行了肝穿刺活检,组织病理学检查提示诊断为小圆细胞瘤。免疫组化染色显示肿瘤中有神经元分化的迹象。综合这些结果,NB 的诊断是正确的。骨髓穿刺和活检结果正常。全身计算机断层扫描显示肝内有一个大肿块,大小为82×70×74毫米,周围轻度强化。偏碘苄基胍(MIBG)闪烁扫描证实了巨大的圆形 MIBG 肝病变,而身体其他部位没有其他明显病变。患者开始接受化疗,经过 4 个疗程的化疗后,超声检查显示肿块缩小至 55×36 毫米。本报告描述了首例儿科原发性肝NB,并提供了详细的临床病理资料。原发性肝NB极为罕见。当遇到组织学表现相似的单发肝肿瘤时,必须考虑神经内分泌肿瘤的可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary Hepatic Neuroblastoma in a 5.5-Month-Old Boy: A Case Report.

The most frequent type of extracranial solid tumor in pediatric cases is neuroblastoma (NB), almost always arising in tissues with sympathetic innervation with only a few reported cases arising in other organs. NBs with hepatic involvement are typically metastatic lesions as primary hepatic NBs are extremely rare. This study presents a 5.5-month-old boy with primary hepatic NB. This case study describes a male 5.5-month-old preterm infant who presented with overt hepatomegaly. Laboratory tests showed an abnormally high level of alpha-fetoprotein. A sonography-guided liver needle biopsy was performed, so histopathological examination suggested the diagnosis of a small round-cell tumor. Immunohistochemical staining demonstrated evidence of neuronal differentiation in the tumor. The sum of these findings was in favor of the diagnosis of NB. Bone marrow aspiration and biopsy were normal. The full-body computed tomography scan revealed a large intrahepatic mass measuring 82×70×74 mm with mild peripheral enhancement. A metaiodobenzylguanidine (MIBG) scintiscan confirmed a huge round MIBG-avid hepatic lesion without other remarkable lesions at other sites in the body. Chemotherapy treatment was started for the patient, and after 4 sessions of chemotherapy, an ultrasound showed that the mass size had decreased to 55×36 mm. This report describes the first primary hepatic NB in a pediatric patient with detailed clinicopathological details. Primary hepatic NB is extremely rare. It is important to consider neuroendocrine tumors as a possibility when faced with a single hepatic tumor that has a similar histological appearance.

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来源期刊
Archives of Iranian Medicine
Archives of Iranian Medicine 医学-医学:内科
CiteScore
4.20
自引率
0.00%
发文量
67
审稿时长
3-8 weeks
期刊介绍: Aim and Scope: The Archives of Iranian Medicine (AIM) is a monthly peer-reviewed multidisciplinary medical publication. The journal welcomes contributions particularly relevant to the Middle-East region and publishes biomedical experiences and clinical investigations on prevalent diseases in the region as well as analyses of factors that may modulate the incidence, course, and management of diseases and pertinent medical problems. Manuscripts with didactic orientation and subjects exclusively of local interest will not be considered for publication.The 2016 Impact Factor of "Archives of Iranian Medicine" is 1.20.
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