Daniel J, Ruzic A, Dalland J, Miller V, Hanna M
Department of Pediatrics, Division of Neonatology, College of Medicine, Univeristy of Kentucky, Lexington, KY, USA.
Department of Surgery, Division of Pediatric Surgery, College of Medicine, University of Kentucky, Lexington, KY, USA.
J Neonatal Perinatal Med. 2017;10(1):113-118. doi: 10.3233/NPM-1617.
Congenital mesoblastic nephroma (CMN) is the most common renal tumor of infancy; however, it occurs infrequently with an incidence of 1 : 125,000. The cellular and classical variants are the most common subtypes of tumors, with a mixed variant occurring infrequently. We describe two cases of mixed variant CMN, which presented within days of each other differing in their clinical behavior. The first case followed a typical course, previously described in the literature, while the other deviated significantly. Traditionally, CMN presents as large abdominal mass in the neonatal period associated with a paraneoplastic syndrome, which can result in hypertension or hypercalcemia. Surgical resection is curative in most cases and long-term prognosis is excellent. Hypertension rarely persists after removal of the tumor, but remained in one of our two patients.
先天性中胚层肾瘤(CMN)是婴儿期最常见的肾肿瘤;然而,其发病率很低,为1∶125000。细胞型和经典型是最常见的肿瘤亚型,混合型很少见。我们描述了两例混合型CMN,这两例在数天内相继出现,临床行为有所不同。第一例病程典型,如先前文献所述,而另一例则有显著差异。传统上,CMN在新生儿期表现为巨大腹部肿块,并伴有副肿瘤综合征,可导致高血压或高钙血症。大多数情况下,手术切除可治愈,长期预后良好。肿瘤切除后高血压很少持续存在,但在我们的两名患者中有一例仍然存在。