Unusual hepatic tumor with features of mesenchymal hamartoma and congenital solitary nonparasitic cyst

We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy,...

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Bibliographic Details
Published in:Pediatric and developmental pathology 2003-05, Vol.6 (3), p.265-269
Main Authors: Azar, Georgia M, Kutin, N, Kahn, Ellen
Format: Article
Language:English
Subjects:
Adolescent
Aneuploidy
Biomarkers - analysis
Cysts - congenital
Cysts - metabolism
Cysts - pathology
Flow Cytometry
Hamartoma - metabolism
Hamartoma - pathology
Hamartoma - surgery
Hepatectomy
Humans
Immunohistochemistry
Karyotyping
Liver Diseases - congenital
Liver Diseases - metabolism
Liver Diseases - pathology
Male
Mucus - metabolism
Tomography, X-Ray Computed
Treatment Outcome
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Description
Summary:We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy, measured 21 cm and was multicystic with solid areas. Microscopically, the cysts were lined by a mucous-producing or intestinal-type epithelium, associated with smooth muscle and small mucous-producing glands. The solid component contained fibrous and adipose tissue, smooth muscle and thick-walled vessels. Aneuploidy was demonstrated by flow cytometry. We interpreted the tumor as having features of a mesenchymal hamartoma and congenital solitary nonparasitic cyst. It is conceivable that the lesions originated with small peribiliary glands with dilatation and intestinal metaplasia.
ISSN:1093-5266
1615-5742
DOI:10.1007/s10024-003-7073-8