Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential.
Détails
ID Serval
serval:BIB_600C9633631A
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential.
Périodique
Human pathology
ISSN
1532-8392 (Electronic)
ISSN-L
0046-8177
Statut éditorial
Publié
Date de publication
08/2016
Peer-reviewed
Oui
Volume
54
Pages
143-151
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Publication Status: ppublish
Résumé
Characteristic but rare vascular neoplasms in the adult liver composed of small vessels with an infiltrative border were collected from an international group of collaborators over a 5-year period (N=17). These tumors were termed hepatic small vessel neoplasm (HSVN), and the histologic differential diagnosis was angiosarcoma (AS). The average age of patients was 54years (range, 24-83years). HSVN was more common in men. The average size was 2.1cm (range, 0.2-5.5cm). Diagnosis was aided by immunohistochemical stains for vascular lineage (CD31, CD34, FLI-1), which were uniformly positive in HSVN. Immunohistochemical stains (p53, c-Myc, GLUT-1, and Ki-67) for possible malignant potential are suggestive of a benign/low-grade tumor. Capture-based next-generation sequencing (using an assay that targets the coding regions of more than 500 cancer genes) identified an activating hotspot GNAQ mutation in 2 of 3 (67%) tested samples, and one of these cases also had a hotspot mutation in PIK3CA. When compared with hepatic AS (n=10) and cavernous hemangioma (n=6), the Ki-67 proliferative index is the most helpful tool in excluding AS, which demonstrated a tumor cell proliferative index greater than 10% in all cases. Strong p53 and diffuse c-Myc staining was also significantly associated with AS but not with HSVN or cavernous hemangioma. There have been no cases with rupture/hemorrhage, disseminated intravascular coagulation, or Kasabach-Merritt syndrome. Thus far, there has been no metastasis or recurrence of HSVN, but complete resection and close clinical follow-up are recommended because the outcome remains unknown.
Pubmed
Web of science
Création de la notice
20/04/2016 14:22
Dernière modification de la notice
20/08/2019 15:17