Second Department of Surgery, Wakayama Medical University Hospital, 811-1 Kimiidera Wakayama, Wakayama, 641-8509, Japan.
BMC Pediatr. 2022 Mar 17;22(1):139. doi: 10.1186/s12887-022-03210-8.
Congenital mesoblastic nephromas mainly present as asymptomatic abdominal masses, but some present hematuria, hypertension or hypercalcemia. Neonatal dyspnea in an early-birth neonate due to rapid tumor growth is reported here for the first time.
A renal tumor and polyhydramnios were detected by ultrasonography of a male fetus at 32 weeks and 3 days of gestation. The mother had abdominal distension due to the polyhydramnios and signs of imminent premature birth. Amniocentesis was performed and the signs of imminent preterm birth subsided, but growth of the renal tumor was noted as a potential cause of respiratory dysfunction. Cesarean section was performed at 36 weeks and 2 days of gestation. His birthweight was 2638 g and his 1 and 5 min APGAR scores were 2 and 4 points, respectively. There was no spontaneous breathing at birth and he had remarkable abdominal distention. He underwent cardiopulmonary resuscitation. After circulation stabilized, emergency surgery was performed because of progressive hypoxemia and respiratory acidosis. Laparotomy revealed a huge tumor arising from the right kidney and right nephrectomy was performed. Histopathological examination led to diagnosis of congenital mesoblastic nephroma. The respiratory condition and circulatory dynamics stabilized after the pressure on the thorax from the tumor was relieved by surgery. The postoperative course was uneventful. No recurrence or complications have been observed in the 36 months since the surgery.
Congenital mesoblastic nephroma can rapidly increase in size from the fetal period and may cause respiratory oncologic emergency, although there is relatively good prognosis.
先天性中胚层肾瘤主要表现为无症状的腹部肿块,但有些表现为血尿、高血压或高钙血症。本报告首次报道了一例因肿瘤快速生长导致早产新生儿出现呼吸困难的病例。
一名男性胎儿在妊娠 32 周 3 天时经超声检查发现肾肿瘤和羊水过多。母亲因羊水过多出现腹胀,并出现早产先兆。行羊膜腔穿刺后早产先兆缓解,但发现肾肿瘤生长,可能导致呼吸功能障碍。妊娠 36 周 2 天行剖宫产术。患儿出生体重为 2638g,1 分钟和 5 分钟时的 Apgar 评分为 2 分和 4 分,出生时无自主呼吸,腹胀明显。予心肺复苏,循环稳定后,患儿出现进行性低氧血症和呼吸性酸中毒,行急诊手术。剖腹探查发现右侧肾脏起源的巨大肿瘤,行右肾切除术。组织病理学检查诊断为先天性中胚层肾瘤。手术解除肿瘤对胸腔的压迫后,呼吸状况和循环动力学稳定,术后过程平稳。术后 36 个月未见复发或并发症。
先天性中胚层肾瘤可在胎儿期迅速增大,可能导致呼吸相关的肿瘤急症,但预后相对较好。