Département de chirurgie viscérale et urologique pédiatrique, Pôle enfant, Hôpital Lapeyronie, 34295 Montpellier, France.
J Pediatr Surg. 2012 Aug;47(8):e21-3. doi: 10.1016/j.jpedsurg.2012.03.090.
Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt.
间叶性肾瘤是迄今为止最常见的肾内胎儿肿瘤。据我们所知，我们报告了首例产前发现的肾内神经母细胞瘤病例。在一名 30 孕周的胎儿中，常规产前超声检查发现肾脏内有回声均匀的肿块，磁共振成像也证实了这一点。这名男婴出生时体重为 3280 克，伴有高血压和心力衰竭。产后超声检查证实左肾内有肿瘤，伴有微钙化和肾周淋巴结病。通过剖腹手术进行了开放性左肾全切除术。病理研究报告称，该肿块为肾内神经母细胞瘤，伴有局部和区域侵犯。术后立即的尿液分析显示香草扁桃酸（VMA）水平很高，血液样本显示去甲肾上腺素水平很高。本报告的目的是证明产前肾内神经母细胞瘤在临床上和影像学上可以模拟间叶性肾瘤。超声检查中出现的高血压、钙化和淋巴结病应提高对可能的恶性过程的怀疑指数。如果诊断有疑问，应在术前检测尿液香草扁桃酸（VMA）和甲氧基肾上腺素。
