Mesenchymal Hamartoma of the Liver in Older Children: An Adult Variant or a Different Entity? Report of a Case With Review of the Literature
| Autor: | Virgone, C, Cecchetto, Giovanni, Dall'Igna, P, Zanon, GIOVANNI FRANCO, Cillo, Umberto, Alaggio, Rita, Virgone, Calogero |
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| Rok vydání: | 2012 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Histology Adolescent Cell of origin Hamartoma Notch signaling pathway Context (language use) Mesenchymal hamartoma Pathology and Forensic Medicine Lesion medicine Basic Helix-Loop-Helix Transcription Factors Humans Receptor Notch2 Child Aged Homeodomain Proteins business.industry Mesenchymal stem cell Liver Neoplasms Middle Aged Phenotype Gene Expression Regulation Neoplastic Medical Laboratory Technology Liver Child Preschool Transcription Factor HES-1 Hepatic tumor Female Bile Ducts medicine.symptom business Signal Transduction |
| Zdroj: | Scopus-Elsevier |
| ISSN: | 1533-4058 |
| Popis: | Mesenchymal hamartoma of the liver (MHL) is an uncommon benign hepatic tumor typically affecting children under 2 years of age. Only 5% of MHL occur after 5 years and are very rarely observed in adults. According to age, MHL may differ in their morphologic features. We report a case of an 11-year-old boy with MHL, resembling a malignant lesion from a clinical point of view, characterized by unusual histologic features: a prominent myxoid stroma, with a minimal ductular component, and absent cystic spaces. The present case and others reported in older children or adults demonstrate that these lesions may represent a potential diagnostic pitfall when occurring outside their classic clinical context especially because of their peculiar histologic findings. Moreover, it may be hypothesized that variation in morphology might be related to different evolutive stages of the cell of origin. To support this hypothesis, we therefore studied the presence of components of the Notch pathway inside and outside the lesion. Their absence inside the tumor and, in contrast, the expression of Notch2 and HES1 evident in overrepresented bile ducts present at the periphery might explain not only the lack of bile ducts, but also indicate a more adult phenotype compared with classic pediatric MHL, which show more bile ducts and liver trabeculae embedded in the mesenchymal matrix. |
| Databáze: | OpenAIRE |
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