Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association

Abdominal tumor masses are a very rare disease in the fetus. The authors present the first reported case of neonatal multicystic adenomatoid hamartoma of the pancreas associated with well-differentiated fetal epithelial subtype hepatoblastoma and reveal clinical, histologic, and imagistic aspects. C...

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Main Authors: Valentin Varlas, Oana Neagu, Andreea Moga, Radu Bălănescu, Roxana Bohiltea, Radu Vladareanu, Laura Balanescu
Format: Article
Language:English
Published: MDPI AG 2022-03-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/12/3/758
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author Valentin Varlas
Oana Neagu
Andreea Moga
Radu Bălănescu
Roxana Bohiltea
Radu Vladareanu
Laura Balanescu
author_facet Valentin Varlas
Oana Neagu
Andreea Moga
Radu Bălănescu
Roxana Bohiltea
Radu Vladareanu
Laura Balanescu
author_sort Valentin Varlas
collection DOAJ
description Abdominal tumor masses are a very rare disease in the fetus. The authors present the first reported case of neonatal multicystic adenomatoid hamartoma of the pancreas associated with well-differentiated fetal epithelial subtype hepatoblastoma and reveal clinical, histologic, and imagistic aspects. Case presentation: A 36-week-old female newborn in whom a 25-week ultrasound showed a relatively homogeneous pancreatic echogenic mass (34 × 30 × 55 mm) with compression of the inferior vena cava and retrograde dilation. Postnatal CT showed a giant pancreatic tumor mass (113 × 70 × 60 mm), with areas enhancing contrast and cystic/necrotic areas and a hypodense, hypocaptive nodule of 8 × 6 mm located at segment IV of the liver; thrombosis of the subhepatic segment of the inferior vena cava and both renal veins. Histopathological and immunohistochemical studies confirmed the diagnosis of multicystic pancreatic adenomatoid hamartoma and well-differentiated fetal epithelial subtype hepatoblastoma. Conclusions: Pancreatic hamartoma can be difficult to diagnose (especially prenatal), with or without nonspecific symptoms. The synchronous presence of hepatoblastoma complicated the therapeutic conduct and prognosis of this case, with the diagnosis being confirmed histopathologically and immunohistochemically after liver biopsy.
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spelling doaj.art-0ae291ace1454865b0ed5b19e0c97fd62023-11-24T00:56:36ZengMDPI AGDiagnostics2075-44182022-03-0112375810.3390/diagnostics12030758Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor AssociationValentin Varlas0Oana Neagu1Andreea Moga2Radu Bălănescu3Roxana Bohiltea4Radu Vladareanu5Laura Balanescu6Department of Obstetrics and Gynecology, Filantropia Clinical Hospital, 011171 Bucharest, RomaniaDepartment of Anatomopathology, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, RomaniaDepartment of Pediatric Surgery, Children Emergency Hospital “Grigore Alexandrescu”, 011743 Bucharest, RomaniaFaculty of General Medicine, “Carol Davila” University of Medicine and Pharmacy, 37 Dionisie Lupu St., 020021 Bucharest, RomaniaDepartment of Obstetrics and Gynecology, Filantropia Clinical Hospital, 011171 Bucharest, RomaniaFaculty of General Medicine, “Carol Davila” University of Medicine and Pharmacy, 37 Dionisie Lupu St., 020021 Bucharest, RomaniaFaculty of General Medicine, “Carol Davila” University of Medicine and Pharmacy, 37 Dionisie Lupu St., 020021 Bucharest, RomaniaAbdominal tumor masses are a very rare disease in the fetus. The authors present the first reported case of neonatal multicystic adenomatoid hamartoma of the pancreas associated with well-differentiated fetal epithelial subtype hepatoblastoma and reveal clinical, histologic, and imagistic aspects. Case presentation: A 36-week-old female newborn in whom a 25-week ultrasound showed a relatively homogeneous pancreatic echogenic mass (34 × 30 × 55 mm) with compression of the inferior vena cava and retrograde dilation. Postnatal CT showed a giant pancreatic tumor mass (113 × 70 × 60 mm), with areas enhancing contrast and cystic/necrotic areas and a hypodense, hypocaptive nodule of 8 × 6 mm located at segment IV of the liver; thrombosis of the subhepatic segment of the inferior vena cava and both renal veins. Histopathological and immunohistochemical studies confirmed the diagnosis of multicystic pancreatic adenomatoid hamartoma and well-differentiated fetal epithelial subtype hepatoblastoma. Conclusions: Pancreatic hamartoma can be difficult to diagnose (especially prenatal), with or without nonspecific symptoms. The synchronous presence of hepatoblastoma complicated the therapeutic conduct and prognosis of this case, with the diagnosis being confirmed histopathologically and immunohistochemically after liver biopsy.https://www.mdpi.com/2075-4418/12/3/758fetal abdominal massfetal pancreatic tumorshamartomahepatoblastomaultrasonographyfetal inferior vena cava anomalies
spellingShingle Valentin Varlas
Oana Neagu
Andreea Moga
Radu Bălănescu
Roxana Bohiltea
Radu Vladareanu
Laura Balanescu
Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
Diagnostics
fetal abdominal mass
fetal pancreatic tumors
hamartoma
hepatoblastoma
ultrasonography
fetal inferior vena cava anomalies
title Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
title_full Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
title_fullStr Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
title_full_unstemmed Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
title_short Fetal Pancreatic Hamartoma Associated with Hepatoblastoma—An Unusual Tumor Association
title_sort fetal pancreatic hamartoma associated with hepatoblastoma an unusual tumor association
topic fetal abdominal mass
fetal pancreatic tumors
hamartoma
hepatoblastoma
ultrasonography
fetal inferior vena cava anomalies
url https://www.mdpi.com/2075-4418/12/3/758
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