title: Telangiectatic focal nodular hyperplasia of the liver in the perinatal period: case report.
abstract: We report a case of congenital telangiectatic focal nodular hyperplasia, a rare variant form of the disease. The patient was a 2-month-old boy whose parents noticed abdominal distention about 2 weeks after birth, and ultrasonogram revealed a large mass in the liver. He underwent right lobectomy, and gross findings showed an ill-defined mass without any central scar. Histologic findings demonstrated proliferating hepatocytes without atypia arranged in cords of 1- or 2-cell thickness with marked sinusoidal dilatation and extramedullary hematopoiesis. In addition, a significantly increased Ki-67 labeling index in the tumor compared with non-tumor liver cells, and cytogenetic analysis of 23 G-banded metaphase preparations revealed 3 abnormal karyotypes, suggesting hyperplastic or neoplastic features. To the best of our knowledge, the present case is only the third documented case of congenital telangiectatic focal nodular hyperplasia.
authors.affiliation: Cyto-histopathology, Graduate School of Medical Science, Kitasato University, 1-15-1 Kitasato Sagamihara, Kanagawa 228-8555, Japan.
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Okamura
authors.forename: Hisaya
authors.initials: H
authors.lastname: Nakadate
authors.forename: Kazuo
authors.initials: K
authors.lastname: Ishida
authors.forename: Saori
authors.initials: S
authors.lastname: Nakahara
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Isobe
authors.forename: Makoto
authors.initials: M
authors.lastname: Ohbu
authors.forename: Isao
authors.initials: I
authors.lastname: Okayasu
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism; D049109:Cell Proliferation; D002869:Chromosome Aberrations; D020732:Cytogenetic Analysis; D019084:Fluorescent Antibody Technique, Indirect; D020518:Focal Nodular Hyperplasia / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D022781:Hepatocytes / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007223:Infant; D019394:Ki-67 Antigen / Q000378:metabolism; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D008297:Male; D013684:Telangiectasis / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D019394:Ki-67 Antigen