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

• 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
• ISSN: 1093-5266; 1615-5742
• DOI: 10.1007/s10024-003-7073-8

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.