Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance

Background: Congenital mesoblastic nephroma represents 3–10% of all pediatric renal tumors. With the advancement of ultrasound diagnostics and magnetic resonance imaging, the diagnosis of this renal neoplasm is increasingly being established prenatally and at birth. It usually presents as a benign t...

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Main Authors: Hristina Zakić, Olivera Kontić Vučinić, Jelena Stamenković, Jovan Jevtić, Milena Perišić Mitrović, Maja Životić
Format: Article
Language:English
Published: MDPI AG 2025-01-01
Series:Biomedicines
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Online Access:https://www.mdpi.com/2227-9059/13/1/196
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author Hristina Zakić
Olivera Kontić Vučinić
Jelena Stamenković
Jovan Jevtić
Milena Perišić Mitrović
Maja Životić
author_facet Hristina Zakić
Olivera Kontić Vučinić
Jelena Stamenković
Jovan Jevtić
Milena Perišić Mitrović
Maja Životić
author_sort Hristina Zakić
collection DOAJ
description Background: Congenital mesoblastic nephroma represents 3–10% of all pediatric renal tumors. With the advancement of ultrasound diagnostics and magnetic resonance imaging, the diagnosis of this renal neoplasm is increasingly being established prenatally and at birth. It usually presents as a benign tumor, but it can severely affect pregnancy outcomes, contributing to perinatal morbidity and mortality. Lissencephaly belongs to a rare category of neurodevelopmental disorders marked by the absence of a substantial reduction in the typical folds and grooves in the cerebral cortex. The prognosis for patients with lissencephaly is extremely poor, carrying with it a high mortality rate. Case Presentation: We present a case of congenital mesoblastic nephroma (CMN) diagnosed with polyhydramnios at 28 weeks of gestation, which led to preterm delivery at 29 weeks and a fatal outcome for the newborn. Histopathological examination confirmed the diagnosis of CMN along with fetal pachygyria/lissencephaly. The aim of this study is to point out the characteristics and unique correlation between CMN and lissencephaly, and to illustrate the histopathological features of CMN and lissencephaly through an educational example derived from our presented index case. To the best to our knowledge, the association of CMN with lissencephaly has not been described in the literature so far. Conclusions: Outlining the prenatal progression of CMN and the outcome of pregnancies involving fetal CMN and lissencephaly, this case underscores the importance of comprehensive ultrasound examinations, including central nervous system evaluation, to identify potential coexisting anomalies and refine prenatal diagnostic practices.
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spelling doaj-art-abe5438809e540dca43128108290d8372025-01-24T13:24:21ZengMDPI AGBiomedicines2227-90592025-01-0113119610.3390/biomedicines13010196Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical SignificanceHristina Zakić0Olivera Kontić Vučinić1Jelena Stamenković2Jovan Jevtić3Milena Perišić Mitrović4Maja Životić5Clinics of Gynecology and Obstetrics, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaClinics of Gynecology and Obstetrics, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaClinics of Gynecology and Obstetrics, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaInstitute of Pathology “Dr. Ðorđe Joannović”, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaClinics of Gynecology and Obstetrics, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaInstitute of Pathology “Dr. Ðorđe Joannović”, Faculty of Medicine, University of Belgrade, 11000 Belgrade, SerbiaBackground: Congenital mesoblastic nephroma represents 3–10% of all pediatric renal tumors. With the advancement of ultrasound diagnostics and magnetic resonance imaging, the diagnosis of this renal neoplasm is increasingly being established prenatally and at birth. It usually presents as a benign tumor, but it can severely affect pregnancy outcomes, contributing to perinatal morbidity and mortality. Lissencephaly belongs to a rare category of neurodevelopmental disorders marked by the absence of a substantial reduction in the typical folds and grooves in the cerebral cortex. The prognosis for patients with lissencephaly is extremely poor, carrying with it a high mortality rate. Case Presentation: We present a case of congenital mesoblastic nephroma (CMN) diagnosed with polyhydramnios at 28 weeks of gestation, which led to preterm delivery at 29 weeks and a fatal outcome for the newborn. Histopathological examination confirmed the diagnosis of CMN along with fetal pachygyria/lissencephaly. The aim of this study is to point out the characteristics and unique correlation between CMN and lissencephaly, and to illustrate the histopathological features of CMN and lissencephaly through an educational example derived from our presented index case. To the best to our knowledge, the association of CMN with lissencephaly has not been described in the literature so far. Conclusions: Outlining the prenatal progression of CMN and the outcome of pregnancies involving fetal CMN and lissencephaly, this case underscores the importance of comprehensive ultrasound examinations, including central nervous system evaluation, to identify potential coexisting anomalies and refine prenatal diagnostic practices.https://www.mdpi.com/2227-9059/13/1/196congenital mesoblastic nephromalissencephalypachygyriaprenatal course
spellingShingle Hristina Zakić
Olivera Kontić Vučinić
Jelena Stamenković
Jovan Jevtić
Milena Perišić Mitrović
Maja Životić
Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
Biomedicines
congenital mesoblastic nephroma
lissencephaly
pachygyria
prenatal course
title Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
title_full Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
title_fullStr Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
title_full_unstemmed Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
title_short Coexisting Congenital Mesoblastic Nephroma and Lissencephaly: Unique Case Report with Pathological Analysis and Its Clinical Significance
title_sort coexisting congenital mesoblastic nephroma and lissencephaly unique case report with pathological analysis and its clinical significance
topic congenital mesoblastic nephroma
lissencephaly
pachygyria
prenatal course
url https://www.mdpi.com/2227-9059/13/1/196
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