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Abstract：
BACKGROUND: Tumors are rare in neonatal age. Congenital mesoblastic nephroma (CMN) is a usually benign renal tumor observed at birth, or in the first months of life. It may also be identified prenatally and associated with polyhydramnios leading to preterm delivery. Effective treatment is surgical in most cases, consisting in total nephrectomy. In literature, very few studies report on the neonatal management of such a rare disease, and even less are those describing its uncommon complications.
METHODS: We report on two single-center newborns affected with CMN. The first patient is a preterm female baby, born at 30+ 1 weeks of gestation (WG) due to premature labor, with prenatal (25 WG) identification of an intra-abdominal fetal mass associated with polyhydramnios. Once obtained the clinical stability, weight gain, instrumental (computed tomography, CT, showing a 4.8 × 3.3 cm left renal neoformation) and histological/molecular characterization of the lesion (renal needle biopsy picture of classic CMN with ETV6-NTRK3 translocation), a left nephrectomy was performed at 5 weeks of chronological age. The following clinical course was complicated by intestinal obstruction due to bowel adherences formation, then by an enterocutaneous fistula, requiring multiple surgical approaches including transitory ileo- and colostomy, before the conclusive anastomoses intervention. The second patient is a 17-day-old male term baby, coming to our observation due to postnatal evidence of palpable left abdominal mass (soon defined through CT, showing a 7.5 × 6.5 cm neoformation in the left renal lodge), feeding difficulties and poor weight gain. An intravenous diuretic treatment was needed due to the developed hypertension and hypercalcemia, which regressed after the nephrectomy (histological diagnosis of cellular CMN with ETV6-NTRK3 fusion) performed at day 26. In neither case was chemotherapy added. Both patients have been included in multidisciplinary follow-up, they presently show regular growth and neuromotor development, normal renal function and no local/systemic recurrences or other gastrointestinal/urinary disorders.
CONCLUSIONS: The finding of a fetal abdominal mass should prompt suspicion of CMN, especially if it is associated with polyhydramnios; it should also alert obstetricians and neonatologists to the risk of preterm delivery. Although being a usually benign condition, CMN may be associated with neonatal systemic-metabolic or postoperative complications. High-level surgical expertise, careful neonatological intensive care and histopathological/cytogenetic-molecular definition are the cornerstones for the optimal management of patients. This should also include an individualized follow-up, oriented to the early detection of any possible recurrences or associated anomalies and to a better quality of life of children and their families.
METHODS: We report on two single-center newborns affected with CMN. The first patient is a preterm female baby, born at 30+ 1 weeks of gestation (WG) due to premature labor, with prenatal (25 WG) identification of an intra-abdominal fetal mass associated with polyhydramnios. Once obtained the clinical stability, weight gain, instrumental (computed tomography, CT, showing a 4.8 × 3.3 cm left renal neoformation) and histological/molecular characterization of the lesion (renal needle biopsy picture of classic CMN with ETV6-NTRK3 translocation), a left nephrectomy was performed at 5 weeks of chronological age. The following clinical course was complicated by intestinal obstruction due to bowel adherences formation, then by an enterocutaneous fistula, requiring multiple surgical approaches including transitory ileo- and colostomy, before the conclusive anastomoses intervention. The second patient is a 17-day-old male term baby, coming to our observation due to postnatal evidence of palpable left abdominal mass (soon defined through CT, showing a 7.5 × 6.5 cm neoformation in the left renal lodge), feeding difficulties and poor weight gain. An intravenous diuretic treatment was needed due to the developed hypertension and hypercalcemia, which regressed after the nephrectomy (histological diagnosis of cellular CMN with ETV6-NTRK3 fusion) performed at day 26. In neither case was chemotherapy added. Both patients have been included in multidisciplinary follow-up, they presently show regular growth and neuromotor development, normal renal function and no local/systemic recurrences or other gastrointestinal/urinary disorders.
CONCLUSIONS: The finding of a fetal abdominal mass should prompt suspicion of CMN, especially if it is associated with polyhydramnios; it should also alert obstetricians and neonatologists to the risk of preterm delivery. Although being a usually benign condition, CMN may be associated with neonatal systemic-metabolic or postoperative complications. High-level surgical expertise, careful neonatological intensive care and histopathological/cytogenetic-molecular definition are the cornerstones for the optimal management of patients. This should also include an individualized follow-up, oriented to the early detection of any possible recurrences or associated anomalies and to a better quality of life of children and their families.
摘要：
背景：肿瘤在新生儿时代是罕见的。先天性中胚层肾瘤（CMN）通常是出生时观察到的良性肾脏肿瘤，或者在生命的头几个月。也可以在产前鉴定，并与羊水过多有关，导致早产。在大多数情况下，有效的治疗是手术，包括全肾切除术。在文学中,很少有研究报告这种罕见疾病的新生儿管理，更少的是那些描述其罕见的并发症。
方法：我们报告了两名受CMN影响的单中心新生儿。第一个病人是一个早产的女婴,出生在妊娠30+1周（WG）由于早产，产前（25WG）鉴定与羊水过多相关的腹内胎儿肿块。一旦获得临床稳定性，体重增加,仪器(计算机断层扫描，CT,显示4.8×3.3厘米的左肾新生）和病变的组织学/分子特征（经典CMN伴ETV6-NTRK3易位的肾针活检照片），左侧肾切除术在5周龄时进行.由于肠粘连形成，以下临床过程并发肠梗阻，然后是肠皮肤瘘，需要多种手术方法，包括短暂回肠和结肠造口术，在决定性的吻合干预之前。第二个病人是一个17天大的男性足月婴儿,由于产后可触及的左腹部肿块的证据（很快通过CT确定，在左肾小屋显示7.5×6.5厘米的新生)，喂养困难和体重增加差。由于高血压和高钙血症的发展，需要静脉利尿剂治疗，在第26天进行肾切除术（具有ETV6-NTRK3融合的细胞CMN的组织学诊断）后消退。在两种情况下都没有添加化疗。两名患者均已纳入多学科随访，它们目前显示有规律的生长和神经运动发育，肾功能正常，无局部/全身复发或其他胃肠道/泌尿系疾病。
结论：发现胎儿腹部肿块应提示CMN的怀疑，特别是如果它与羊水过多有关;它也应该提醒产科医生和新生儿专家早产的风险。虽然通常是良性疾病，CMN可能与新生儿全身代谢或术后并发症有关。高水平的外科专业知识，仔细的新生儿重症监护和组织病理学/细胞遗传学-分子定义是患者最佳管理的基石.这还应包括个性化的后续行动，旨在及早发现任何可能的复发或相关异常，并提高儿童及其家庭的生活质量。
方法：我们报告了两名受CMN影响的单中心新生儿。第一个病人是一个早产的女婴,出生在妊娠30+1周（WG）由于早产，产前（25WG）鉴定与羊水过多相关的腹内胎儿肿块。一旦获得临床稳定性，体重增加,仪器(计算机断层扫描，CT,显示4.8×3.3厘米的左肾新生）和病变的组织学/分子特征（经典CMN伴ETV6-NTRK3易位的肾针活检照片），左侧肾切除术在5周龄时进行.由于肠粘连形成，以下临床过程并发肠梗阻，然后是肠皮肤瘘，需要多种手术方法，包括短暂回肠和结肠造口术，在决定性的吻合干预之前。第二个病人是一个17天大的男性足月婴儿,由于产后可触及的左腹部肿块的证据（很快通过CT确定，在左肾小屋显示7.5×6.5厘米的新生)，喂养困难和体重增加差。由于高血压和高钙血症的发展，需要静脉利尿剂治疗，在第26天进行肾切除术（具有ETV6-NTRK3融合的细胞CMN的组织学诊断）后消退。在两种情况下都没有添加化疗。两名患者均已纳入多学科随访，它们目前显示有规律的生长和神经运动发育，肾功能正常，无局部/全身复发或其他胃肠道/泌尿系疾病。
结论：发现胎儿腹部肿块应提示CMN的怀疑，特别是如果它与羊水过多有关;它也应该提醒产科医生和新生儿专家早产的风险。虽然通常是良性疾病，CMN可能与新生儿全身代谢或术后并发症有关。高水平的外科专业知识，仔细的新生儿重症监护和组织病理学/细胞遗传学-分子定义是患者最佳管理的基石.这还应包括个性化的后续行动，旨在及早发现任何可能的复发或相关异常，并提高儿童及其家庭的生活质量。
