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

  • Gill RM University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143. Electronic address: ryan.gill@ucsf.edu.
  • Buelow B University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143.
  • Mather C University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143.
  • Joseph NM University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143.
  • Alves V Universidade de Sao Paulo, Department of Pathology, Sao Paulo, Brazil, 05508-090.
  • Brunt EM Washington University, Department of Pathology and Immunology, St Louis, MO, United States, 63110.
  • Liu TC Washington University, Department of Pathology and Immunology, St Louis, MO, United States, 63110.
  • Makhlouf H NCI, NIH, Rockville, MD, United States, 20850.
  • Marginean C Ottawa Hospital, Department of Pathology, Ottawa, Ontario, Canada, K1H 8L6.
  • Nalbantoglu I Washington University, Department of Pathology and Immunology, St Louis, MO, United States, 63110.
  • Sempoux C Institut Universitaire de Pathologie, CHUV, Lausanne, Switzerland, CH-1011.
  • Snover DC Fairview Southdale Hospital, Edina, MN, 55435; Department of Laboratory Medicine and Pathology, The University of Minnesota Medical School, Minneapolis, MN, 55435.
  • Thung SN Mount Sinai Health System, Department of Pathology, NY, New York, United States, 10029.
  • Yeh MM University. of Washington, Department of Pathology, Seattle, Washington, United States, 98195.
  • Ferrell LD University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143.
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  • 2016-04-20
Published in:
  • Human pathology. - 2016
Angiosarcoma
GNAQ
Hemangioma
Hepatic small vessel neoplasm
Liver
Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Cell Proliferation
Class I Phosphatidylinositol 3-Kinases
DNA Mutational Analysis
Diagnosis, Differential
Female
GTP-Binding Protein alpha Subunits, Gq-G11
Hemangioma, Cavernous
Hemangiosarcoma
Humans
Immunohistochemistry
Ki-67 Antigen
Liver Neoplasms
Male
Middle Aged
Mutation
Neoplasm Grading
Phosphatidylinositol 3-Kinases
Predictive Value of Tests
Proto-Oncogene Proteins c-myc
Terminology as Topic
Tumor Suppressor Protein p53
Vascular Neoplasms
Young Adult
English 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.
Language
  • English
Open access status
green
Identifiers
Persistent URL
https://sonar.ch/global/documents/126113
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