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表现为威尔姆斯瘤并伴有下腔静脉血栓形成及肺转移的神经母细胞瘤:病例系列报道

Neuroblastoma presenting like a Wilms' tumor with thrombus in inferior vena cava and pulmonary metastases: a case series.

作者信息

Gaetan Genevieve, Ouimet Alain, Lapierre Chantale, Teira Pierre, Sartelet Herve

机构信息

Surgery Department, Hôpital Sainte Justine, Montréal, Canada.

Medical Imaging Department, Hôpital Sainte Justine, Montréal, Canada.

出版信息

Springerplus. 2014 Jul 10;3:351. doi: 10.1186/2193-1801-3-351. eCollection 2014.

Abstract

Neuroblastomas and Wilms' tumors are frequent pediatric solid tumors. The first is frequently detected in the adrenal gland and the second develops in the kidneys. The extension through the vena cava and the lung metastases are frequent in Wilms' tumors and are rarely seen in neuroblastoma. We present the cases of three children with abdominal tumors with thrombus in the inferior vena cava and pulmonary metastases demonstrating a stage 4 neuroblastoma. The three male patients were between 23 to 48 months old. They presented an abdominal mass, near the superior pole of the kidney. Thrombus of the vena cava was showed on imaging studies in all cases and pulmonary metastases were always found. Catecholamine metabolites were present in the first case and negative in the two others. Two out of three patients had a radical nephrectomy. The pathological analysis always found a poorly differentiated or undifferentiated neuroblastoma without MYCN amplification and confirmed the vein tumoral thrombus in the second case. The evolution of the first two patients was unfavorable and the third is alive. Invasion of the inferior vena cava and pulmonary metastases in children with neuroblastoma is uncommon and can modify the surgical management.

摘要

神经母细胞瘤和肾母细胞瘤是常见的小儿实体瘤。前者常于肾上腺被发现,后者则在肾脏发生。肾母细胞瘤常出现经腔静脉扩展及肺转移,而神经母细胞瘤中则少见。我们报告了3例患有腹部肿瘤的儿童病例,其下腔静脉有血栓形成且伴有肺转移,表现为4期神经母细胞瘤。这3例男性患者年龄在23至48个月之间。他们均表现为腹部肿块,位于肾上级附近。所有病例的影像学检查均显示有腔静脉血栓形成,且均发现有肺转移。第1例患者儿茶酚胺代谢产物呈阳性,另2例呈阴性。3例患者中有2例行根治性肾切除术。病理分析始终发现为低分化或未分化神经母细胞瘤,无MYCN扩增,且在第2例中证实有静脉肿瘤血栓形成。前2例患者病情进展不利,第3例仍存活。神经母细胞瘤患儿出现下腔静脉侵犯和肺转移并不常见,且可能会改变手术治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f955/4112030/6495aa43481b/40064_2013_1059_Fig1_HTML.jpg

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