Multiple Hepatoblastomas Associated with Trisomy 18 in a 3-Year-Old Girl

A very rare case of full trisomy 18 associated with multiple hepatoblastomas is reported. The patient also had ventricular septal defect and patent ductus arteriosus, which were repaired at 6 months of age. After the cardiac surgery, she was noted to have an abdominal mass and an elevated serum α-fe...

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Bibliographic Details
Published in:Pediatric hematology and oncology 1997, Vol.14 (5), p.463-467
Main Authors: Teraguchi, Masayuki, Nogi, Shunji, Ikemoto, Yumiko, Ogino, Hirotaro, Kohdera, Urara, Sakaida, Noriko, Okamura, Akiharu, Hamada, Yoshinori, Kobayashi, Yohnosuke
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - genetics
Biological and medical sciences
Child, Preschool
Chromosomes, Human, Pair 18
Female
Gastroenterology. Liver. Pancreas. Abdomen
hepatoblastoma
Hepatoblastoma - genetics
Humans
Infant, Newborn
Karyotyping
Liver Neoplasms - genetics
Liver Neoplasms - pathology
Liver Neoplasms - physiopathology
Liver Neoplasms - surgery
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Medical sciences
Neoplasms, Multiple Primary - genetics
Neoplasms, Multiple Primary - pathology
Neoplasms, Multiple Primary - physiopathology
Neoplasms, Multiple Primary - surgery
Tracheal Neoplasms - genetics
Tracheal Neoplasms - pathology
Tracheal Neoplasms - physiopathology
Tracheal Neoplasms - surgery
Trisomy
trisomy 18
Tumors
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Summary:A very rare case of full trisomy 18 associated with multiple hepatoblastomas is reported. The patient also had ventricular septal defect and patent ductus arteriosus, which were repaired at 6 months of age. After the cardiac surgery, she was noted to have an abdominal mass and an elevated serum α-fetoprotein level. A partial hepatic lobectomy was performed at 7 months of age, and the resected tumor was diagnosed as a fetal-type hepatoblastoma. At 2 years and 4 months of age, a chest radiograph disclosed an elevated left diaphragm, and abdominal ultrasonography demonstrated a tumor in the left hepatic lobe. The resected tumor was also diagnosed as a fetal-type hepatoblastoma. Chromosomal analysis demonstrated that the karyotypes of peripheral blood and hepatic tumor cells obtained on two occasions were both 47,XX, +18. She has no evidence of recurrence at 3 years of age without specific therapy.
ISSN:0888-0018
1521-0669
DOI:10.3109/08880019709028777