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Arch Iran Med. 2020;23(9): 621-623.
doi: 10.34172/aim.2020.73

Scopus ID: 85091810837
  Abstract View: 179
  PDF Download: 155

Case Report

A Case of Congenital Hepatoblastoma Coexisting with Pulmonary Hypertension

Zeynep Canan Özdemir 1*, Adviye Çakıl Sağlık 2, Yeter Düzenli Kar 1, Pelin Köşger 3, Deniz Arık 4, Ayşe Neslihan Tekin 2, Özcan Bör 1

1 Division of Pediatric Hematology/Oncology, Department of Pediatrics, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
2 Division of Neonatology, Department of Pediatrics, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
3 Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
4 Department of Pathology, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
*Corresponding Author: Zeynep Canan Özdemir, MD; Division of Pediatric Hematology/Oncology, Department of Pediatrics, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, 26480, Turkey. Tel: +90-505-669 1140; Fax: +90-222-239 3450; Email: efecanan@yahoo.com

Abstract

Hepatoblastoma (HBL) is the most frequently detected malignant tumor of the liver in childhood. HBLs detected antenatally or up to 3 months after birth are considered congenital HBLs. We report a five-day-old female infant in whom a hepatic mass was detected at 20 weeks’ gestation. At birth (36 weeks), the hepatic mass measured 12x6 cm, and she had respiratory distress. Pulmonary hypertension (PHT) was detected on echocardiographic evaluation. Despite dual medical therapy, her PHT did not improve. Histologically, the biopsy demonstrated a mixed epithelial-mesenchymal HBL with predominance of fetal morphology in the epithelial component. Chemotherapy was initiated on postnatal day 15; however, the baby died of respiratory failure on postnatal day 23. Conclusion: HBL is an embryonal tumor which can develop early in the intrauterine period. Although the mechanism is not known, it may cause PHT which would affect the prognosis negatively.
Keywords: Congenital, Hepatoblastoma, Pulmonary hypertension
Cite this article as: Özdemir ZC, Çakıl Sağlık A, Düzenli Kar Y, Köşger P, Arık D, Tekin AN, et al. A case of congenital hepatoblastoma coexisting with pulmonary hypertension. Arch Iran Med. 2020;23(9):621–623. doi: 10.34172/aim.2020.73.
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Submitted: 19 Nov 2018
Accepted: 16 Mar 2019
ePublished: 01 Sep 2020
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