Skull vault hemangioma
Updates to Article Attributes
Skull vault haemangiomas (SVH), or haemangiomas of the calvaria, are benign slow-growing vascular neoplasms affecting the skull diploe in any location.
Please refer to the article on intraosseous haemangiomas for a general discussion of that entity.
Epidemiology
The calvaria is the second most prevalent site for intraosseous haemangiomas, particularly the frontal and parietal bones 1,. It represents 10% of benign neoplasms of the skull 4 and represents 0.2% of all osseous tumours 2. Commonly occurs in women in the 4th-5th decades of life with a 3:1 female-male ratio 4.
Clinical presentation
These tumours are slow-growing and are generally asymptomatic, unless when palpable due to a lump produced by an expansion of the outer table 3.
Pathology
As an intraosseous haemangioma, SVH are classified as venous, cavernous, or capillary type, according to their predominant vascular network. Histologically, it demonstrates hamartomatous vascular tissue within endothelium, but may also contain fat, smooth muscle, fibrous tissue, and thrombus 1-3.
Radiographic features
Plain radiograph
Lytic lesion with a sclerotic rim (usually with a honeycomb- or sunburst-like appearance).
CT
Usually presents as an expansile bone lesion with thin borders and a characteristic sunburst pattern of trabecular thickening radiating from a common centre. Erosions of both internal or external plates can occur and may be associated with internal or external tumour expansion. Sometimes, bony trabeculae can grow beyond the corticla bone, and simulate an agressive pseudo-"hair-on-end" periosteal reaction 4.
Angiography (DSA)
DSA is important for the surgical planning of smaller lesions and embolisation of larger ones.
MRI
Signal intensity is somewhat variable, depending largely on the amount of fat content4.
-
T1:
non-homogeneously hypo- to isointense signalTypically hyperintense, though atypical T1 hypointense forms ar enot rare. - T2/FLAIR: hyperintense signal
- T1 C+ (Gd): Homogeneous enhancement is often present
Treatment and prognosis
Treatment isusually not always necessary and. Rare indications include: mass effect, haemorrhage control, and aesthetic improvement. Treatment options are:
- radiation therapy
- embolisation to reduce intraoperative blood loss
- surgical resection
- intralesional ethanol injection
-<p><strong>Skull vault haemangiomas (SVH)</strong>, or <strong>haemangiomas of the calvaria</strong>, are benign slow-growing vascular neoplasms affecting the skull diploe in any location. </p><p>Please refer to the article on <a href="/articles/primary-intraosseous-haemangioma">intraosseous haemangiomas</a> for a general discussion of that entity. </p><h4>Epidemiology</h4><p>The calvaria is the second most prevalent site for intraosseous haemangiomas, particularly the frontal and parietal bones <sup>1</sup>, and represents 0.2% of all osseous tumours <sup>2</sup>. Commonly occurs in women in the 4th-5th decades of life.</p><h4>Clinical presentation</h4><p>These tumours are slow-growing and are generally asymptomatic, unless when palpable due to a lump produced by an expansion of the outer table <sup>3</sup>. </p><h4>Pathology</h4><p>As an intraosseous haemangioma, SVH are classified as venous, cavernous, or capillary type, according to their predominant vascular network. Histologically, it demonstrates hamartomatous vascular tissue within endothelium, but may also contain fat, smooth muscle, fibrous tissue, and thrombus <sup>1-3</sup>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Lytic lesion with a sclerotic rim (usually with a honeycomb- or sunburst-like appearance).</p><h5>CT</h5><p>Usually presents as an expansile bone lesion with thin borders and a characteristic sunburst pattern of trabecular thickening radiating from a common centre. Erosions of both internal or external plates can occur and may be associated with internal or external tumour expansion. </p><h5>Angiography (DSA)</h5><p>DSA is important for the surgical planning of smaller lesions and embolisation of larger ones. </p><h5>MRI</h5><p>Signal intensity is somewhat variable, depending largely on the amount of fat content. </p><ul>- +<p><strong>Skull vault haemangiomas (SVH)</strong>, or <strong>haemangiomas of the calvaria</strong>, are benign slow-growing vascular neoplasms affecting the skull diploe in any location. </p><p>Please refer to the article on <a href="/articles/primary-intraosseous-haemangioma">intraosseous haemangiomas</a> for a general discussion of that entity. </p><h4>Epidemiology</h4><p>The calvaria is the second most prevalent site for intraosseous haemangiomas, particularly the frontal and parietal bones <sup>1</sup>. It represents 10% of benign neoplasms of the skull <sup>4</sup> and 0.2% of all osseous tumours <sup>2</sup>. Commonly occurs in women in the 4th-5th decades of life with a 3:1 female-male ratio <sup>4</sup>.</p><h4>Clinical presentation</h4><p>These tumours are slow-growing and are generally asymptomatic, unless when palpable due to a lump produced by an expansion of the outer table <sup>3</sup>. </p><h4>Pathology</h4><p>As an intraosseous haemangioma, SVH are classified as venous, cavernous, or capillary type, according to their predominant vascular network. Histologically, it demonstrates hamartomatous vascular tissue within endothelium, but may also contain fat, smooth muscle, fibrous tissue, and thrombus <sup>1-3</sup>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Lytic lesion with a sclerotic rim (usually with a honeycomb- or sunburst-like appearance).</p><h5>CT</h5><p>Usually presents as an expansile bone lesion with thin borders and a characteristic sunburst pattern of trabecular thickening radiating from a common centre. Erosions of both internal or external plates can occur and may be associated with internal or external tumour expansion. Sometimes, bony trabeculae can grow beyond the corticla bone, and simulate an agressive pseudo-"hair-on-end" periosteal reaction <sup>4</sup>.</p><h5>Angiography (DSA)</h5><p>DSA is important for the surgical planning of smaller lesions and embolisation of larger ones. </p><h5>MRI</h5><p>Signal intensity is somewhat variable, depending largely on the amount of fat content <sup>4</sup>. </p><ul>
-<strong>T1:</strong> non-homogeneously hypo- to isointense signal</li>- +<strong>T1:</strong> Typically hyperintense, though atypical T1 hypointense forms ar enot rare. </li>
-<strong>T1 C+ (Gd):</strong> enhancement is often present</li>-</ul><h4>Treatment and prognosis </h4><p>Treatment is not always necessary and indications include: mass effect, haemorrhage control, and aesthetic improvement. Treatment options are:</p><ul>- +<strong>T1 C+ (Gd):</strong> Homogeneous enhancement is often present</li>
- +</ul><h4>Treatment and prognosis </h4><p>Treatment usually not necessary. Rare indications include: mass effect, haemorrhage control, and aesthetic improvement. Treatment options are:</p><ul>
References changed:
- 4. Albert Pons Escoda, Pablo Naval Baudin, Paloma Mora et-al. Imaging of skull vault tumors in adults. (2020) Insights into Imaging. 11 (1): 1. <a href="https://doi.org/10.1186/s13244-019-0820-9">doi:10.1186/s13244-019-0820-9</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32056014">Pubmed</a> <span class="ref_v4"></span>
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