Primary Bone Vascular Neoplasms: An Integrated Biological Perspective from Bone Pathology to Precision Oncology
4 affiliations
- 1Department of Biomedical Sciences, School of Health Sciences, International Hellenic University, Sindos, 57400 Thessaloniki, Greece
- 2Orthopaedic Department, University General Hospital of Alexandroupolis, 68100 Alexandroupolis, Greece
- 3Department of Pathology, Faculty of Medicine, School of Health Sciences, University of Ioannina, 45500 Ioannina, Greece
- 4Department of Anatomy, Histology and Embryology, School of Medicine, University of Patras, 26504 Patras, Greece
Abstract
Primary bone vascular neoplasms comprise a rare and biologically heterogeneous group of endothelial tumors encompassing benign, intermediate, and malignant entities with distinct clinicopathological, molecular, and prognostic characteristics. These tumors remain diagnostically and biologically challenging because of overlapping histopathological features, heterogeneous clinical behavior, evolving molecular landscapes, and limited disease-specific evidence. Advances in bone pathology, immunophenotyping, molecular diagnostics, and genomic profiling have substantially improved tumor classification and biological characterization. Importantly, contemporary classification and molecular studies have also highlighted the biological heterogeneity of vascular lesions involving bone, distinguishing well-defined vascular neoplasms from vascular malformations and identifying additional molecularly defined entities relevant to osseous disease and differential diagnosis. Concurrently, increasing recognition of the bone microenvironment and osteoimmunology provides a broader framework for understanding interactions among endothelial, skeletal, stromal, and immune populations and the extracellular matrix, with potential relevance to angiogenesis, bone remodeling, and tumor biology. Previous authoritative syntheses have established the classification, molecular features, diagnosis, and clinical management of these neoplasms. However, comparatively limited attention has been devoted to integrating tumor-intrinsic alterations with vascular–bone crosstalk, extracellular-matrix and mechanobiological signaling, osteoimmune regulation, and emerging precision-oncology strategies. Building on these foundations, this review integrates current evidence on classification, molecular pathogenesis, the skeletal microenvironment, osteoimmunology, biomarkers, and precision oncology. Where relevant, molecularly defined vascular neoplasms and vascular anomalies involving bone are discussed to clarify classification, molecular context, and differential diagnosis without conflating these lesions with the principal WHO-defined primary vascular neoplasms of bone. Findings directly demonstrated in primary bone vascular neoplasms are distinguished from mechanistic concepts extrapolated from physiological bone biology, other bone tumors, or non-osseous vascular neoplasms. Through this evidence-calibrated approach, we propose an integrated vascular–bone–immune framework, identify key knowledge gaps, and define priorities for disease-specific translational research and more individualized patient management.
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BibTeX
@article{anetaki2026primary,
title = {{Primary Bone Vascular Neoplasms: An Integrated Biological Perspective from Bone Pathology to Precision Oncology}},
author = {Aikaterini Anetaki and Constantine Anetakis and Ioannis Chaniotakis and Christos Mpogiatzis and Anna Goussia and Dionysios J. Papachristou and Mattheos Bobos},
journal = {Cancers},
year = {2026},
month = oct,
doi = {10.3390/cancers18193229},
url = {https://europepmc.org/article/PMC/PMC13664260},
note = {CC BY},
}
RIS
TY - JOUR
TI - Primary Bone Vascular Neoplasms: An Integrated Biological Perspective from Bone Pathology to Precision Oncology
AU - Aikaterini Anetaki
AU - Constantine Anetakis
AU - Ioannis Chaniotakis
AU - Christos Mpogiatzis
AU - Anna Goussia
AU - Dionysios J. Papachristou
AU - Mattheos Bobos
PY - 2026
DA - 2026/10/01
JO - Cancers
DO - 10.3390/cancers18193229
UR - https://europepmc.org/article/PMC/PMC13664260
AB - Primary bone vascular neoplasms comprise a rare and biologically heterogeneous group of endothelial tumors encompassing benign, intermediate, and malignant entities with distinct clinicopathological, molecular, and prognostic characteristics. These tumors remain diagnostically and biologically challenging because of overlapping histopathological features, heterogeneous clinical behavior, evolving molecular landscapes, and limited disease-specific evidence. Advances in bone pathology, immunophenotyping, molecular diagnostics, and genomic profiling have substantially improved tumor classification and biological characterization. Importantly, contemporary classification and molecular studies have also highlighted the biological heterogeneity of vascular lesions involving bone, distinguishing well-defined vascular neoplasms from vascular malformations and identifying additional molecularly defined entities relevant to osseous disease and differential diagnosis. Concurrently, increasing recognition of the bone microenvironment and osteoimmunology provides a broader framework for understanding interactions among endothelial, skeletal, stromal, and immune populations and the extracellular matrix, with potential relevance to angiogenesis, bone remodeling, and tumor biology. Previous authoritative syntheses have established the classification, molecular features, diagnosis, and clinical management of these neoplasms. However, comparatively limited attention has been devoted to integrating tumor-intrinsic alterations with vascular–bone crosstalk, extracellular-matrix and mechanobiological signaling, osteoimmune regulation, and emerging precision-oncology strategies. Building on these foundations, this review integrates current evidence on classification, molecular pathogenesis, the skeletal microenvironment, osteoimmunology, biomarkers, and precision oncology. Where relevant, molecularly defined vascular neoplasms and vascular anomalies involving bone are discussed to clarify classification, molecular context, and differential diagnosis without conflating these lesions with the principal WHO-defined primary vascular neoplasms of bone. Findings directly demonstrated in primary bone vascular neoplasms are distinguished from mechanistic concepts extrapolated from physiological bone biology, other bone tumors, or non-osseous vascular neoplasms. Through this evidence-calibrated approach, we propose an integrated vascular–bone–immune framework, identify key knowledge gaps, and define priorities for disease-specific translational research and more individualized patient management.
SN - 2072-6694
ER -