A rare cause of neonatal hypertension: Congenital mesoblastic nephroma

Soyaltın, Eren; Alaygut, Demet; Alparslan, Caner; Özdemir, Tunç; Mutlubaş, Fatma; Kasap-Demir, Belde; Yavaşcan, Önder

doi:10.24953/turkjped.2018.02.014

Cited by 5 publications

(4 citation statements)

References 13 publications

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“…In literature, both complications are reported associated with CMN, due to secretion of prostaglandins, parathyroid hormone, parathyroid-related and glucagon-like peptides, as well as renin released by tumor cells. Both conditions required medical therapy with furosemide and were promptly resolved after tumor removal, highlighting that their pathogenesis is related with factors secreted by tumor cells (paraneoplastic syndromes) [ 21 , 29 , 34 ]. The underlying pathophysiological mechanism of hypertension is the secondary hyperaldosteronism, due to increased plasma renin activity (PRA); therefore, serum electrolytes and acid-base equilibrium (ABE) should be monitored, owing to the higher risk of hypokalemia and metabolic alkalosis.…”

Section: Discussionmentioning

confidence: 99%

“…The underlying pathophysiological mechanism of hypertension is the secondary hyperaldosteronism, due to increased plasma renin activity (PRA); therefore, serum electrolytes and acid-base equilibrium (ABE) should be monitored, owing to the higher risk of hypokalemia and metabolic alkalosis. Furosemide, which was used in both our patients, is considered as the first choice drug in the treatment of hypercalcemia and hypertension, due to its effect in promoting urinary calcium excretion; in case of refractory hypertension, co-administration of calcium channel blockers (e.g., nifedipine and amlodipine) may be considered more appropriate [ 29 ].…”

Section: Discussionmentioning

confidence: 99%

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Report and follow-up on two new patients with congenital mesoblastic nephroma

Serra,

Cimador,

Giuffrè

et al. 2023

Ital J Pediatr

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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. Cases presentation 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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Report and follow-up on two new patients with congenital mesoblastic nephroma

Serra,

Cimador,

Giuffrè

et al. 2023

Ital J Pediatr

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“…Also, associations with a prevalent side localization have been previously described, but data are con icting (Table 1). [21,30,35]. The underlying pathophysiological mechanism of hypertension is the secondary hyperaldosteronism, due to increased plasma renin activity (PRA); therefore, serum electrolytes and acidbase equilibrium (ABE) should be monitored, owing to the higher risk of hypokalemia and metabolic alkalosis [30].…”

Section: Discussionmentioning

confidence: 99%

Report and follow-up on two new patients with Congenital Mesoblastic Nephroma

Serra

Cimador

Giuffrè

et al. 2023

Preprint

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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. Cases presentation 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. After that clinical stability, weight gain, instrumental (computed tomography, CT, showing a 4.8 x 3.3 cm left renal neoformation) and histological/molecular characterization of the lesion (renal needle biopsy picture of classic CMN with ETV6-NTRK3 translocation) were obtained, 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, and 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 x 6.5 cm neoformation in the left renal lodge), feeding difficulties, and poor weight gain. He then developed hypertension and hypercalcemia, needing intravenous diuretic treatment and which regressed after 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, and 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, and alert obstetricians and neonatologists also for 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 early detection of any possible recurrences or associated anomalies, and to improve the quality of life of these children and their families.

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“…Mesoblastic nephroma is the most frequent type of NWRT in the neonatal period and 90% percent of patients are seen under the age of 3 months old. Total resection of the tumor is usually curative, but local recurrences can be seen [11][12][13]. Cellular variants and high mitotic indexes of mesoblastic nephroma have poor outcomes with bone and brain metastases and local recurrence [12,13].…”

Section: Discussionmentioning

confidence: 99%

Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

et al. 2019

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Background/aim: Non-Wilms renal tumors (NWRTs) are rarely encountered in children. The aim of this study is to determine the treatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey. Materials and methods: Medical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995-2018) were reviewed retrospectively. Results: There was male predominance (female/male: 7/13); the median age at diagnosis was 3.2 years old (0.1-13.5 years old). The major histological groups included mesoblastic nephroma (MBN), (n: 5, 25%), malignant rhabdoid tumor (MRT), (n: 5, 25%), renal cell carcinoma, (n: 3, 15%), inflammatory myofibroblastic tumor (n: 2, 10%), multilocular cystic renal tumors (n: 2, 10%), metanephric adenoma (n: 1, 5%), renal neuroblastoma (n: 1, 5%), and bilateral renal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) (n: 1, 5%). All of the patients with NWRTs had radical nephrectomy except the child with bilateral renal ES/PNET. Six children died because of progressive disease; the mortality rate was 30% (n: 6). Conclusion: We have made the first report of bilateral renal involvement of ES/PNET in the English medical literature. Physicians dealing with pediatric renal masses should be alert to the high mortality rate in children with MRT, MBN, and ES/PNET and they should design substantial management plans for NWRTs.

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A rare cause of neonatal hypertension: Congenital mesoblastic nephroma

Cited by 5 publications

References 13 publications

Report and follow-up on two new patients with congenital mesoblastic nephroma

Report and follow-up on two new patients with congenital mesoblastic nephroma

Report and follow-up on two new patients with Congenital Mesoblastic Nephroma

Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

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