Poggi Giovanni Maria, Fognani Giuliana, Cuzzubbo Daniela, Liguori Antonio, Resti Massimo, Pela Ivana
Pediatric Clinic Unit, Department of Sciences for Woman and Child's Health, University of Florence, Florence, Italy.
Case Rep Oncol. 2011 May;4(2):400-5. doi: 10.1159/000331211. Epub 2011 Aug 18.
Neuroblastoma is the most common extracranial solid tumor in childhood. Its presenting signs and symptoms may be highly variable, depending on the location of the primary tumor and its local or metastatic diffusion and, rarely, with paraneoplastic syndrome such as opsoclonus-myoclonus-ataxia syndrome and gastrointestinal disturbances, due to autoantibodies or to aberrant secretion of vasoactive intestinal peptide. Herein we describe a 10-month-old child with neuroblastoma presenting with a complex clinical picture characterized by acute kidney injury manifested by renal insufficiency and signs and symptoms of tubulointerstitial damage, with polyuria, polydipsia, glucosuria, aminoaciduria and hypochloremic metabolic alkalosis, and of glomerular damage with heavy proteinuria. Imaging study documented a suprarenal mass enveloping the aorta and its abdominal and renal ramifications and bilaterally renal veins. This clinical picture shows some analogies with the hyponatremic-hypertensive syndrome concerning the renovascular disease; however, in absence of systemic arterial hypertension, the heavy proteinuria and the polyuria could be explained by sectional increased intraglomerular pressure, due to local renal blood vessels constriction. Hypochloremic metabolic alkalosis probably developed because of local production of renin, responsible of renin-angiotensin-aldosterone system activation, but above all because of chloride loss through sweating. The long lasting dehydration, due to vomiting, sweating and polyuria, caused prolonged prerenal failure evolving in proximal tubular damage manifestations.
神经母细胞瘤是儿童期最常见的颅外实体瘤。其临床表现可能差异很大，这取决于原发肿瘤的位置及其局部或转移扩散情况，很少情况下会出现副肿瘤综合征，如眼阵挛-肌阵挛-共济失调综合征和胃肠道紊乱，这是由自身抗体或血管活性肠肽的异常分泌引起的。在此，我们描述了一名10个月大的神经母细胞瘤患儿，其临床表现复杂，以急性肾损伤为特征，表现为肾功能不全以及肾小管间质损伤的体征和症状，包括多尿、烦渴、糖尿、氨基酸尿和低氯性代谢性碱中毒，同时伴有严重蛋白尿的肾小球损伤。影像学检查显示一个肾上腺肿块，包绕主动脉及其腹部和肾分支以及双侧肾静脉。这种临床表现与肾血管疾病的低钠血症-高血压综合征有一些相似之处；然而，在没有系统性动脉高血压的情况下，严重蛋白尿和多尿可能是由于局部肾血管收缩导致肾小球内压力局部升高所致。低氯性代谢性碱中毒可能是由于局部肾素产生，导致肾素-血管紧张素-醛固酮系统激活，但最重要的是由于出汗导致氯丢失。由于呕吐、出汗和多尿导致的长期脱水，引起了持续时间较长的肾前性肾衰竭，并发展为近端肾小管损伤的表现。
