ScholarLens Back to search
1970•PEDIATRICSRequires access

EPITHELIAL HEPATOBLASTOMA ASSOCIATED WITH CONGENITAL HEMIHYPERTROPHY AND CYSTATHIONINURIA: PRESENTATION OF A CASE

Clementina F. Geiser, Arantxa Medina Baez, Schindler Am, Vivian E. Shih

Open publisher page 50 citations

Abstract

A 13-month-old boy with hepatoblastoma and hemihypertrophy is presented; this is the fifth known case with this association. Histological study showed that hemihypertrophy was related to cellular hyperplasia but not to hypertrophy of individual cells. Karyotypes of leukocytes and of skin fibroblasts from both sides of the body were normal. The association of hemihypertrophy and hepatoblastoma stresses the dysontogenetic origin of this neoplasm and signals the possibility of this tumor in children with asymmetry. Our patient showed cystathioninuria, which was related to the high content of cystathionine in his tumor. The presence of cystathioninuria in 7 of 10 patients with hepatoblastoma so far examined, shows the usefulness of urinary amino acid screening in the diagnosis of this rare childhood neoplasm.

About this research paper

What this paper is about

A 13-month-old boy with hepatoblastoma and hemihypertrophy is presented; this is the fifth known case with this association. Histological study showed that hemihypertrophy was related to cellular hyperplasia but not to hypertrophy of individual cells. Karyotypes of leukocytes and of skin fibroblasts from both sides of the body were normal. The association of hemihypertrophy and hepatoblastoma stresses the dysontogenetic origin of this neoplasm and signals the possibility of this tumor in children with asymmetry. Our patient showed cystathioninuria, which was related to the high content of cystathionine in his tumor. The presence of cystathioninuria in 7 of 10 patients with hepatoblastoma so far examined, shows the usefulness of urinary amino acid screening in the diagnosis of this rare childhood neoplasm.

Why it matters

OpenAlex reports 50 citations for this work. Citation counts describe recorded attention and do not establish research quality.

Key contribution

A contribution statement is not available in the OpenAlex record.

Method / approach

Method details are not available in the OpenAlex metadata.

Main findings

Findings are not separately available in the OpenAlex metadata.

Limitations

Limitations are not available in the OpenAlex metadata.

Applications

Application details are not available in the OpenAlex metadata.

Available abstract

A 13-month-old boy with hepatoblastoma and hemihypertrophy is presented; this is the fifth known case with this association. Histological study showed that hemihypertrophy was related to cellular hyperplasia but not to hypertrophy of individual cells. Karyotypes of leukocytes and of skin fibroblasts from both sides of the body were normal. The association of hemihypertrophy and hepatoblastoma stresses the dysontogenetic origin of this neoplasm and signals the possibility of this tumor in children with asymmetry. Our patient showed cystathioninuria, which was related to the high content of cystathionine in his tumor. The presence of cystathioninuria in 7 of 10 patients with hepatoblastoma so far examined, shows the usefulness of urinary amino acid screening in the diagnosis of this rare childhood neoplasm.

Key concepts: Hemihypertrophy, Hepatoblastoma, Medicine, Wilms' tumor, Hyperplasia, Pathology, Presentation (obstetrics), Muscle hypertrophy

Related papers

Related paper 1Related paper 2Related paper 3
Back to paper searchBrowse research topicsOriginal source
EPITHELIAL HEPATOBLASTOMA ASSOCIATED WITH CONGENITAL HEMIHYPERTROPHY AND CYSTATHIONINURIA: PRESENTATION OF A CASE — Research Paper | ScholarLens