Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential

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• 作者：Ryan M. Gill ; MD ; PhD^a ; ^{ryan.gill@ucsf.edu" class="auth_mail" title="E-mail the corresponding author} ; Benjamin Buelow ; MD ; PhD^a ; Cheryl Mather ; MD^a ; Nancy M. Joseph ; MD ; PhD^a ; Venancio Alves ; MD ; PhD^b ; Elizabeth M. Brunt ; MD^c ; Ta-Chiang Liu ; MD ; PhD^c ; Hala Makhlouf ; MD^d ; Celia Marginean ; MD^e ; ILKe Nalbantoglu ; MD^c ; Christine Sempoux ; MD ; PhD^f ; Dale C. Snover ; MD^g ; ^h ; Swan N. Thung ; MDⁱ ; Matthew M. Yeh ; MD ; PhD^j ; Linda D. Ferrell ; MD^a
• 关键词：Liver ; Hemangioma ; Angiosarcoma ; Hepatic small vessel neoplasm ; GNAQ
• 刊名：Human Pathology
• 出版年：2016
• 出版时间：August 2016
• 年：2016
• 卷：54
• 期：Complete
• 页码：143-151
• 全文大小：2973 K

文摘

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 54 years (range, 24-83 years). HSVN was more common in men. The average size was 2.1 cm (range, 0.2-5.5 cm). 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.