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[Hepatic mesenchymal hamartoma in children. Immunohistochemical, ultrastructural and flow cytometric case study]
E Justrabo1, L Martin, N Yaziji
1Laboratoire d'Anatomie Pathologique, Faculté de Médecine, Dijon.
Insights
A rare liver lesion, mesenchymal hamartoma, was identified in an infant with elevated alphafetoprotein. This tumor contained biliary/hepatic cells and showed DNA aneuploidy, indicating potential for abnormal cell growth.
Area of Science:
- Pediatric Pathology
- Hepatobiliary Diseases
- Tumor Biology
Background:
- Mesenchymal hamartoma is a rare, benign liver lesion typically presenting in infancy.
- Differentiating mesenchymal hamartoma from malignant liver tumors is crucial for appropriate management.
- High serum alphafetoprotein levels can be associated with certain pediatric liver lesions.
Observation:
- A 7-month-old girl presented with high serum alphafetoprotein levels and a liver lesion.
- Histopathological examination revealed a mesenchymal hamartoma composed of loose connective tissue with epithelial cells of biliary or hepatic origin.
- Immunohistochemistry confirmed cytokeratins 7 and 19 in bile duct epithelium.
Findings:
- Ultrastructural analysis demonstrated well-differentiated ductal structures within a myxoid mesenchyme, with cyst formation.
- Flow cytometry revealed DNA aneuploidy in the lesion's nuclei, with a DNA index of 1.28.
- The combination of histological features and DNA aneuploidy provides insights into the tumor's nature.
Implications:
- This case highlights the importance of comprehensive histopathological and cytogenetic analysis in diagnosing pediatric liver lesions.
- Understanding the cellular composition and genetic characteristics of mesenchymal hamartoma aids in distinguishing it from other liver tumors.
- Further research into the biological behavior of DNA aneuploid mesenchymal hamartomas is warranted.
Abstract:
Mesenchymal hamartoma is a rare liver lesion. This lesion was found in a 7-month-old girl with high serum alphafaetoprotein serum levels and was composed of loose connective tissue containing a certain number of epithelial cells of biliary or hepatic origin. Immunohistochemical studies showed that cytokeratins 7 and 19 were localized in bile duct epithelium. The ultrastructural study showed that the hamartoma was composed of well differentiated ductal structures surrounded by a myxoid mesenchyma with cysts formed either from degenerative mesenchymal areas or from dilated ducts. Flow cytometric analysis of nuclei from frozen tissue revealed that the lesion was DNA aneuploid, with a DNA index of 1.28.