scholarly journals Surgical management of cellular congenital mesoblastic nephroma in a neonate: a case report and review of literature

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Ali Ihab ◽  
Al Otaibi Abdullah ◽  
Abduldaem Abdullah ◽  
Alonazi Mohammad ◽  
Nojoom Maha

Abstract Background Congenital mesoblastic nephroma (CMN) is the most common type of renal stromal tumor in neonates. It is classified into cellular, classical, and mixed types. The multidisciplinary management approach is the mainstay of management. We are reporting a case of neonatal congenital mesoblastic nephroma in a full-term boy with intrauterine growth restriction and hypertension managed effectively in our institution. Case presentation A full-term boy with intrauterine growth restriction (IUGR) with a birth weight of 2.3 kg, referred at birth with an abdominal mass. His antenatal scan at 35 weeks showed a cystic abdominal mass. On delivery, a huge visible abdominal mass of cystic consistency and smooth surface was noticed at the right side of the abdomen. blood pressure was 98/75 mmHg. It was responsive to hydralazine. Aldosterone and renin were significantly elevated at more than 100 and 500 ng/dl, respectively. Serum neurone-specific enolase (NSE) was 35 ng/ml while alfa feto protein was (AFP) 50,000 kIU/L. An abdominal ultrasound scan revealed an ill-defined large heterogeneous mass of 6.09 × 6.5 × 5.77 cm that arises from the right kidney. A computed tomography scan confirmed a right kidney mass with peripheral claw sign of the normal right renal tissue. It was crossing the midline and causing a mass effect on the adjacent structures. The right renal artery and vein were compressed and shifted posteriorly and inferomedially. Right radical nephrectomy was performed via a right lower transverse incision. The tumor was completely excised with a tumor weight of 270 g and a size of 10.5 × 8 × 5.5 cm. Histopathological diagnosis was mesoblastic nephroma of a cellular type which was confirmed by immunohistochemistry. Post-operatively, the blood pressure has significantly reduced and antihypertensive medications were eventually weaned off. The multidisciplinary team decided to preserve chemotherapy only in case of recurrence. At 3 months follow-up, no features of recurrence were noticed based on surveillance ultrasonography. Conclusion Mesoblastic nephroma should be considered in any newborn with renal mass. The multidisciplinary team approach with aggressive management of hypertension, detailed radiological investigation, and complete tumor resection is fundamental for obtaining an excellent outcome for such entities.

2016 ◽  
Vol 311 (2) ◽  
pp. F312-F319 ◽  
Author(s):  
John Henry Dasinger ◽  
Suttira Intapad ◽  
Miles A. Backstrom ◽  
Anthony J. Carter ◽  
Barbara T. Alexander

Placental insufficiency programs an increase in blood pressure associated with a twofold increase in serum testosterone in male growth-restricted offspring at 4 mo of age. Population studies indicate that the inverse relationship between birth weight and blood pressure is amplified with age. Thus, we tested the hypothesis that intrauterine growth restriction programs an age-related increase in blood pressure in male offspring. Growth-restricted offspring retained a significantly higher blood pressure at 12 but not at 18 mo of age compared with age-matched controls. Blood pressure was significantly increased in control offspring at 18 mo of age relative to control counterparts at 12 mo; however, blood pressure was not increased in growth-restricted at 18 mo relative to growth-restricted counterparts at 12 mo. Serum testosterone levels were not elevated in growth-restricted offspring relative to control at 12 mo of age. Thus, male growth-restricted offspring no longer exhibited a positive association between blood pressure and testosterone at 12 mo of age. Unlike hypertension in male growth-restricted offspring at 4 mo of age, inhibition of the renin-angiotensin system with enalapril (250 mg/l for 2 wk) did not abolish the difference in blood pressure in growth-restricted offspring relative to control counterparts at 12 mo of age. Therefore, these data suggest that intrauterine growth restriction programs an accelerated age-related increase in blood pressure in growth-restricted offspring. Furthermore, this study suggests that the etiology of increased blood pressure in male growth-restricted offspring at 12 mo of age differs from that at 4 mo of age.


2010 ◽  
Vol 22 (8) ◽  
pp. 1188 ◽  
Author(s):  
Revati A. Darp ◽  
Hendrina A. de Boo ◽  
Hui Hui Phua ◽  
Mark H. Oliver ◽  
José G. B. Derraik ◽  
...  

Intrauterine growth restriction (IUGR) has life-long health implications, yet there is no effective prenatal treatment. Daily intra-amniotic administration of insulin-like growth factor (IGF)-1 to IUGR fetal sheep improves fetal gut maturation but suppresses hepatic igf1 gene expression. Fetal hepatic blood supply is regulated, in part, by shunting of oxygen- and nutrient-rich umbilical venous blood through the ductus venosus, with the left hepatic lobe predominantly supplied by umbilical venous blood and the right hepatic lobe predominantly supplied by the portal circulation. We hypothesised that: (1) once-weekly intra-amniotic IGF-1 treatment of IUGR would be effective in promoting gut maturation; and (2) IUGR and its treatment with intra-amniotic IGF-1 would differentially affect igf1 and igf1r mRNA expression in the two hepatic lobes. IUGR fetuses received 360 µg IGF-1 or saline intra-amniotically once weekly from 110 until 131 days gestation. Treatment of IUGR fetuses with IGF-1 reversed impaired gut growth. In unembolised, untreated control fetuses, igf1 mRNA levels were 19% lower in the right hepatic lobe than in the left; in IUGR fetuses, igf1 and igf1r mRNA levels were sixfold higher in the right lobe. IGF-1 treatment reduced igf1 and igf1r mRNA levels in both lobes compared with IUGR fetuses. Thus, weekly intra-amniotic IGF-1 treatment, a clinically feasible approach, reverses the impaired gut development seen in IUGR. Furthermore, igf1 and igf1r mRNA levels are differentially expressed in the two hepatic lobes and relative expression in the two lobes is altered by both IUGR and intra-amniotic IGF-1 treatment.


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