A Challenging Case of Hepatoblastoma Concomitant with Autosomal Recessive Polycystic Kidney Disease and Caroli Syndrome-Review of the Literature

Nevil Kadakia; Steven J Lobritto; Nadia Ovchinsky; Helen E Remotti; Darrell J Yamashiro; Jean C Emond; Mercedes Martinez

doi:10.3389/fped.2017.00114

A Challenging Case of Hepatoblastoma Concomitant with Autosomal Recessive Polycystic Kidney Disease and Caroli Syndrome-Review of the Literature

Front Pediatr. 2017 Jun 7:5:114. doi: 10.3389/fped.2017.00114. eCollection 2017.

Authors

Nevil Kadakia¹, Steven J Lobritto¹, Nadia Ovchinsky², Helen E Remotti³, Darrell J Yamashiro^{1

3

4}, Jean C Emond⁴, Mercedes Martinez¹

Affiliations

¹ Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY, United States.
² Department of Pediatrics, Children's Hospital of Montefiore, Bronx, NY, United States.
³ Department of Pathology and Cell Biology, Columbia University College of Physicians and Surgeons, New York, NY, United States.
⁴ Department of Surgery, Columbia University College of Physicians and Surgeons, New York, NY, United States.

Abstract

We report a rare case of an 18-month-old female with autosomal recessive polycystic kidney disease, Caroli syndrome, and pure fetal type hepatoblastoma. The liver tumor was surgically resected with no chemotherapy given. Now 9 years post resection she demonstrates no local or distant recurrence and stable renal function.

Keywords: Caroli syndrome; autosomal recessive polycystic kidney disease; congenital hepatic fibrosis; hepatoblastoma; hepatorenal fibrocystic diseases.

Publication types

Case Reports