Bone & joints

Vascular tumors

Hemangioma


Editorial Board Member: Farres Obeidin, M.D.
Deputy Editor-in-Chief: Borislav A. Alexiev, M.D.
Lucas F. Abrahao Machado, M.D., Ph.D.

Last author update: 21 January 2025
Last staff update: 21 January 2025

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PubMed Search: Hemangioma

Lucas F. Abrahao Machado, M.D., Ph.D.
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Cite this page: Abrahao Machado LF. Hemangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonehemangioma.html. Accessed April 1st, 2025.
Definition / general
Essential features
Terminology
  • Hemangioma; conventional hemangioma; intraosseous hemangioma
  • Gorham-Stout syndrome (also known as vanishing bone disease): diffuse angiomatosis with massive osteolysis (Skeletal Radiol 2000;29:63)
ICD coding
  • ICD-O: 9120/0 - hemangioma, NOS
  • ICD-11
    • EE6Y - other specified fibromatous disorders of skin and soft tissue
    • 2E81.0Y & XH5AW4 - neoplastic hemangioma of other specified site & hemangioma, NOS
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
  • Tissue sampling is the gold standard for a definitive diagnosis (J Orthop Surg Res 2024;19:310)
  • Essential diagnostic criteria according to the WHO classification of tumors: bone tumor with compatible imaging; thin walled blood vessels lined by a single layer of nonatypical endothelial cells
Radiology description
Radiology images

Images hosted on other servers:
Medullary hemangioma in the tibia (Xray)

Medullary hemangioma in the tibia (Xray)

Vertebral hemangioma (CT and MRI)

Vertebral hemangioma (CT and MRI)

Aggressive vertebral hemangioma (MRI)

Aggressive vertebral hemangioma (MRI)

Spinal MRI shows nodular lesion in the vertebral body

Spinal MRI shows nodular lesion in the vertebral body

Lumbar CT showing a vertebral hemangioma

Lumbar CT showing a vertebral hemangioma

Prognostic factors
Case reports
Treatment
  • Asymptomatic, incidentally detected lesions require no treatment (Skeletal Radiol 2000;29:63)
  • In symptomatic tumors, different interventions might be used (Crit Rev Oncol Hematol 2024;195:104268)
    • Curettage and bone grafting
    • Endovascular embolization
    • Percutaneous vertebroplasty
    • Transpedicular ethanol injection
    • Radiation therapy
Gross description
  • Dark red or brown, sponge-like appearance
  • Tumor is usually centered in medullary cavity (Semin Diagn Pathol 2014;31:30)
  • Bone sclerosis and periosteal reaction may be present
Gross images

Contributed by Lucas F. Abrahao Machado, M.D., Ph.D.
Hemangioma of the proximal femur

Hemangioma of the proximal femur

Cavernous hemangioma of the femur

Cavernous hemangioma of the femur

Microscopic (histologic) description
  • Proliferation of thin walled, blood filled vessels lined by a single layer of flat, cytologically bland endothelial cells
  • Usually of capillary or cavernous type
  • Vessels surround pre-existing bone trabeculae and permeate the medullary canal
  • Lumina may be filled with red blood cells, although they are frequently empty or contain only proteinaceous material
  • Variable amounts of surrounding loose connective tissue and fat
  • Secondary changes: thrombosis, calcification, papillary endothelial hyperplasia, reactive sclerosis of involved bone trabeculae
  • References: Skeletal Radiol 2000;29:63, Semin Diagn Pathol 2014;31:30, Surg Pathol Clin 2021;14:645
Microscopic (histologic) images

Contributed by Lucas F. Abrahao Machado, M.D., Ph.D.
Vascular channels around bone trabeculae

Vascular channels around bone trabeculae

Capillary type

Capillary type

Vessels filled with red blood cells

Vessels filled with red blood cells

Vessels with empty lumina

Vessels with empty lumina


Cavernous type Cavernous type

Cavernous type

Surrounding stroma

Surrounding stroma

Thrombosis

Thrombosis

CD31

CD31

ERG

ERG

Molecular / cytogenetics description
  • Specific genetic alterations of hemangioma of bone are still under investigation
  • NFATC1 and NFATC2 fusions (mostly with EWSR1) have been described in few hemangiomas of bone, although they will most likely soon be considered a distinct group of vascular neoplasms (Am J Surg Pathol 2013;37:613, Genes Chromosomes Cancer 2021;60:762, Am J Surg Pathol 2024;48:487)
    • Hemangiomas with NFATC related fusions show epithelioid phenotype and cytologic atypia, with potential for local aggressive behavior and recurrence
Sample pathology report
  • Lesion in thoracic vertebra, biopsy:
    • Benign vascular lesion, consistent with hemangioma (see comment)
    • Comment: The tumor shows a proliferation of thin walled capillary vessels lined by a single layer of nonatypical endothelial cells, coursing through the marrow spaces and encasing pre-existing bony trabeculae. Hemangioma of bone is the most common vascular tumor of bone and asymptomatic or incidentally found cases generally do not require treatment. Radiological and clinical correlation is recommended to confirm the diagnosis.
Differential diagnosis
  • Reactive process:
    • Fewer blood vessels
    • Vascular channels not as haphazardly arranged as in hemangioma
    • Often shows other reactive changes such as fibrosis, inflammation and woven bone
  • Epithelioid hemangioma:
    • Endothelial cells are large, epithelioid and have abundant eosinophilic cytoplasm
    • Intracytoplasmic vacuoles are commonly observed
    • Tumor cells can grow in solid cords and sheets
    • Stroma may contain eosinophils and plasma cells
    • FOSB positivity in the majority of cases (Int J Surg Pathol 2023;31:280)
  • Epithelioid hemangioendothelioma:
    • Epithelioid endothelial cells arranged in cords or clusters in myxohyaline stroma
    • Intracytoplasmic vacuoles are prominent
    • Vascular lumina are usually not observed
    • CAMTA1 positivity in the majority of cases (Am J Surg Pathol 2016;40:94)
  • Angiosarcoma:
    • Tumor cells show a greater degree of atypia
    • Blood vessels demonstrate irregular and complex architecture, sometimes with anastomosing channels
    • Cells often grow in solid clusters
    • Tumor can cause bone destruction and necrosis of surrounding trabeculae
Board review style question #1
Which of the following is one of the most common tumors of the vertebral column and is rarely clinically significant?

  1. Conventional hemangioma
  2. Enchondroma
  3. Epithelioid hemangioma
  4. Plasmacytoma
Board review style answer #1
A. Conventional hemangioma. Hemangioma is the most common type of vascular tumor of the skeleton and one of the most common tumors of vertebrae in general. However, the vast majority are asymptomatic; clinically significant tumors account for < 1% of primary bone tumors. Answer D is incorrect because, in plasmacytoma, the vertebrae are more commonly involved as part of a diffuse process (multiple myeloma) by which the bone marrow is affected; therefore, it is usually a symptomatic and clinically significant tumor. Answer C is incorrect because epithelioid hemangioma is an uncommon variant of hemangioma and the majority arises in long tubular bones. Answer B is incorrect because enchondroma of the spine is very rare. Chondroid lesions occurring in the vertebrae should be carefully investigated as they are generally aggressive, if they are not reactive or metaplastic in nature.

Comment Here

Reference: Hemangioma
Board review style question #2

A 34 year old man underwent biopsy of a lesion in the third lumbar vertebra found incidentally after an abdominal CT scan. Microscopically, as shown in the image above, the lesion revealed numerous vascular channels lined by a single layer of endothelial cells without nuclear atypia, interspersing the pre-existing trabeculae. What is the diagnosis?

  1. Angiosarcoma
  2. Epithelioid hemangioendothelioma
  3. Epithelioid hemangioma
  4. Hemangioma of bone
Board review style answer #2
D. Hemangioma of bone. The image and microscopic description depict a hemangioma of bone, a benign and generally asymptomatic vascular tumor. Answer B is incorrect because epithelioid hemangioendothelioma usually presents with symptoms of pain and swelling and it is histologically characterized by epithelioid cells arranged in cords or clusters within a distinctive myxohyaline stroma, without the formation of vascular lumina. Answer A is incorrect because angiosarcoma usually arises as an ill defined and destructive mass, showing atypical endothelial cells lining irregular vascular channels. Answer C is incorrect because epithelioid hemangioma has a different morphology from conventional hemangioma, revealing larger, epithelioid cells with abundant eosinophilic cytoplasm.

Comment Here

Reference: Hemangioma
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