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Primary hepatic B-cell lymphoma in a child
M H Collins1, A Orazi, M Bauman
1Department of Pathology, James Whitcomb Riley Hospital for Children, Indiana University, School of Medicine, Indianapolis 46202-5200.
Insights
Primary hepatic lymphoma is rare in children. This case highlights a unique calcified B-cell lymphoma in a 12-year-old boy, featuring specific genetic markers and extensive calcification.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Malignancies
- Hematologic Malignancies
Background:
- Primary hepatic lymphoma is an uncommon malignancy in adults and exceedingly rare in pediatric populations.
- Liver tumors in children necessitate a broad differential diagnosis, including rare neoplastic entities.
Observation:
- A 12-year-old male presented with hepatomegaly and jaundice.
- Imaging revealed a calcified intrahepatic mass.
- The mass was diagnosed as a large-cell lymphoma of B-cell origin.
Findings:
- The tumor exhibited bcl-2 protein expression and a near-tetraploid karyotype with t(8;14) and a homogeneously staining region (HSR).
- This represents the fourth documented case of primary hepatic lymphoma in a child and the first with an HSR prior to treatment.
- It is also the first reported human lymphoma with t(8;14) translocation and bcl-2 protein expression.
- Extensive tumor calcification, visualized via computed tomography, is a highly unusual feature for lymphoma.
Implications:
- This case underscores the importance of considering lymphoma in the differential diagnosis of pediatric liver tumors, particularly when serum alpha-fetoprotein levels are normal.
- The unique genetic and radiographic features contribute to the understanding of rare hepatic malignancies.
- Further research into the pathogenesis and treatment of primary hepatic lymphoma in children is warranted.
Abstract:
Lymphoma arising in the liver is uncommon in adults and rare in children. A 12-year-old boy with hepatomegaly and jaundice had a calcified intrahepatic large-cell lymphoma of B-cell origin that expressed bcl-2 protein and had near-tetraploid chromosome number with a t(8;14) (q24;q32) and a homogeneously staining region (HSR). This tumor, only the fourth example of primary hepatic lymphoma in a child, has the rare finding of an HSR before treatment and is the first human lymphoma with t(8;14) that expresses bcl-2 protein. In addition, the demonstration of extensive calcification in the tumor by computed tomography scan is highly unusual for lymphoma. Lymphoma must be considered in the differential diagnosis of primary liver tumors in children and adults, especially if the serum alpha-fetoprotein level is normal.
