Bone & joints

Vascular tumors


Last author update: 1 June 2005
Last staff update: 31 January 2022

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PubMed search: angiosarcoma [title] bone

Nat Pernick, M.D.
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Cite this page: Pernick N. Angiosarcoma. website. Accessed November 29th, 2023.
Definition / general
  • Also called hemangioendothelioma in bone, although angiosarcomas have more cytologic atypia
  • Rare; may be multicentric
  • 1/3 affect long tubular bones but any bone may be affected
  • 1/3 are multifocal, usually in one geographic area, such as an entire leg
  • After diagnosis, search for multicentricity
  • Distant metastases common, often to lungs
  • Graded 1 - 3 based on atypia of endothelial cells
  • Grade 1 have excellent prognosis versus poor prognosis for grade 3
Radiology description
  • Lytic areas of destruction, with minimal / no reactive new bone formation
Gross description
  • Red, hemorrhagic
Gross images

Contributed by Mark R. Wick, M.D.

Primary bone multifocal

Microscopic (histologic) description
  • Obvious atypia of tumor cells, solid areas alternating with irregular, anastomosing vascular channels
  • Necrosis and hemorrhage, brisk mitotic activity
  • Variable differentiation often within same tumor; may be epithelioid or histiocytic
  • May have benign giant cells, eosinophils, occasionally reactive bone formation
Microscopic (histologic) images

Contributed by Mark R. Wick, M.D. and AFIP images

Primary bone

Grade 3

Electron microscopy description
  • Endothelial cell features, may have pericytic features
Additional references
Epithelioid angiosarcoma
Definition / general
  • 80% male, mean age 62 years, range 26 - 83 years
  • 60% multifocal
  • Aggressive clinical course
  • > 90% of tumor cells have epithelioid features

Radiology images

Contributed by Mark R. Wick, M.D.

Primary bone Xray

Case reports

Gross description
  • Friable, hemorrhagic, destructive tumor, 2 - 12 cm
  • Poorly defined, infiltrates medullary canal, frequently erodes cortex and invades adjacent soft tissue

Microscopic (histologic) description
  • Solid and infiltrative sheets replacing the marrow and encasing bony trabeculae
  • No lobular growth pattern
  • Usually with prominent vascular channels or cystically dilated spaces
  • Tumor cells are large, polygonal with abundant eosinophilic cytoplasm, large nuclei with open chromatin, prominent eosinophilic nuclei
  • Frequent intratumoral hemorrhage, neutrophils, intracytoplasmic lumina
  • Frequent mitotic figures and necrosis
  • May have rhabdoid or spindled features

Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.

Various images

Primary bone

Positive stains

Negative stains

Electron microscopy description
  • Long junctions, intracytoplasmic filaments, mitochondria, rough endoplasmic reticulum
  • May contain rare Weibel-Palade bodies

Differential diagnosis
  • Metastatic carcinoma: no well formed vascular channels, no neutrophils; negative for factor VIII, CD31 and CD34
  • Mucin+ cytoplasmic vacuoles

Additional references
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