Vascular Malformation, Primary Intraosseous

Alternative Names

  • Vascular Malformation Osseous
  • VMOS
  • Hemangioma, Intraosseous
  • IOH
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WHO-ICD-10 version:2010

Neoplasms

Benign neoplasms

OMIM Number

606893

Description

Intraosseous hemangioma (IOH) is an extremely rare anomaly, characterized by slow-growing benign vascular malformations, usually in the vertebral column and skull. IOH accounts for less than 1% of all tumors affecting the bone. Most frequently affected bones include the mandible, maxilla, the vertebral column, and the calvaria. Histologically, the hemangiomas can be classified as venous, cavernous, or capillary type, according to their vascular network. The cavernous type is an enlarged mass of blood-filled sinusoidal channels which erodes and displaces normal tissues. The capillary type of hemangioma, on the other hand, consists of multiple tufts of capillaries in radiating loops. The most common form, however, is a mixed form, consisting of both cavernous and capillary type of hemangiomas. This condition is seen more in women than in men, with a ration of 3:1. The most common age of presentation is in the fourth and fifth decades of life.

In most cases, the condition remains asymptomatic, and in a lot of the cases, the finding of the tumors is incidental. Diagnosis is best done with the help of CT or MRI scan. Differential diagnosis includes aneurismal bone cyst, histiocytosis-x, fibrous dysplasia, dermoid and epidermoid cysts, meningioma, osteoma, and osteogenic sarcoma. Confirmation of diagnosis can only be done upon biopsy. The choice of treatment is surgery involving en bloc resection, and establishment of normal bony margins by drill cutterage. Malignant transformation has been observed very rarely, and is reported only after radiation therapy. Therefore, radiotherapy is not recommended.

Molecular Genetics

Although most reported cases of IOH have been sporadic, at least two consanguineous families have been reported to have multiple cases of affected patients. In these patients, homozygosity mapping was able to exclude the TEK Tyrosine Kinase gene, responsible for multiple cutaneous venous malformation 1, Glomulin gene, responsible for glomuvenous malformations, KRIT1 gene, causing cerebral cavernous malformations, and the Endoglin gene.

Epidemiology in the Arab World

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Other Reports

Morocco

Ajja et al. (2005) reported the case of a 31 year-old female, with a progressive left parietal mass. A plain X-ray of the skull and cranial CT-scan showed an osteolytic lesion in the left parietal bone. Surgery consisted of total resection of the lesion and cranioplasty. The postoperative course was uneventful. Histological study revealed a cavernous hemangioma of the diploe.

Oman

Sharma et al. (1999) reported a case of cryptic hemangioma of the superior orbital rim of the right frontal bone in a 43-year old Omani male. This patient presented with a six months history of pain over the right forehead and vertex, which was initially mild and occurring four to five times per day, but then increased in frequency (ten to 15 times/day) and intensity (lancinating pains), and triggered by touching the supraorbital region with no pain between the episodes. These episodes did not respond to an anticonvulsant. Clinical examination was unremarkable and there was no sensory impairment over the forehead or vertex. CT scan of the brain revealed no abnormality, and a diagnosis of trigeminal neuralgia affecting the ophthalmic division was made. While the patient was awaiting posterior fossa decompression, he noticed a subtle bony swelling over the right supraorbital rim which appeared as a cryptic osteolytic lesion with no marginal sclerosis over the supraorbital region in the right frontal bone in plain X-rays. On CT scan, it appeared as a bulge in the right supraorbital rim, while bone window CT scan revealed a small area of smooth expansion of both the inner and the outer tables of the superior orbital rim, suggesting a primary benign intradiploic lesion. The supraorbital region was explored through a transverse right eyebrow incision, and the intraosseous mass was totally excised after being separated from the stretched supraorbital nerves. Histopathological examination of this lesion confirmed the diagnosis of intraosseous hemangioma. The patient had uneventful postoperative period and was asymptomatic over a follow up period of three years.

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