Skull vault hemangiomas (SVH), or hemangiomas of the calvaria, are benign slow-growing vascular neoplasms affecting the skull diploe in any location.
Please refer to the article on intraosseous hemangiomas for a general discussion of that entity.
The calvaria is the second most prevalent site for intraosseous hemangiomas, particularly the frontal and parietal bones 1. It represents 10% of benign neoplasms of the skull 4 and 0.2% of all osseous tumors 2. Commonly occurs in women in the 4th-5th decades of life with a 3:1 female-male ratio 4.
These tumors are slow-growing and are generally asymptomatic, unless when palpable due to a lump produced by an expansion of the outer table 3.
As an intraosseous hemangioma, skull vault hemangiomas 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.
Lytic lesion with a sclerotic rim (usually with a honeycomb- or sunburst-like appearance).
Usually presents as an expansile bone lesion with thin borders and a characteristic sunburst pattern of trabecular thickening radiating from a common center. Erosions of both internal or external plates can occur and may be associated with internal or external tumor expansion. Sometimes, bony trabeculae can grow beyond the cortical bone, and simulate an aggressive pseudo-"hair-on-end" periosteal reaction 4.
DSA is important for the surgical planning of smaller lesions and embolization of larger ones.
Signal intensity is somewhat variable, depending largely on the amount of fat content 4.
- T1: typically hyperintense, though atypical T1 hypointense forms are not rare.
- T2/FLAIR: hyperintense signal
- T1 C+ (Gd): homogeneous enhancement is often present
Treatment and prognosis
Treatment usually not necessary. Rare indications include: mass effect, hemorrhage control, and aesthetic improvement. Treatment options are:
- radiation therapy
- embolization to reduce intraoperative blood loss
- surgical resection
- intralesional ethanol injection
- 1. Gourin CG, Millay DJ. Pathology forum: quiz case 3. Diagnosis: cavernous hemangioma of the nasal bones. Arch. Otolaryngol. Head Neck Surg. 2000;126 (7): 902, 906-7. Pubmed citation
- 2. Reis BL, Carvalho GT, Sousa AA et-al. Primary hemangioma of the skull. Arq Neuropsiquiatr. 2009;66 (3A): 569-71. Pubmed citation
- 3. Politi M, Romeike BF, Papanagiotou P et-al. Intraosseous hemangioma of the skull with dural tail sign: radiologic features with pathologic correlation. AJNR Am J Neuroradiol. 2005;26 (8): 2049-52. Pubmed citation
- 4. Albert Pons Escoda, Pablo Naval Baudin, Paloma Mora, Mònica Cos, Javier Hernandez Gañan, José A. Narváez, Carles Aguilera, Carles Majós. Imaging of skull vault tumors in adults. (2020) Insights into Imaging. 11 (1): 1. doi:10.1186/s13244-019-0820-9 - Pubmed
- 5. Classic imaging features of calvarial hemangioma-a case report. (2018) The Egyptian Journal of Radiology and Nuclear Medicine. 49 (3): 663. doi:10.1016/j.ejrnm.2018.04.010
- 6. D Bastug, O Ortiz, S S Schochet. Hemangiomas in the calvaria: imaging findings. (2013) AJR. American journal of roentgenology. 164 (3): 683-7. doi:10.2214/ajr.164.3.7863894 - Pubmed