An unusual case of intraosseous vascular malformation of the maxilla mimicking fibrous dysplasia: a case report and literature review on imaging features of intraosseous vascular anomalies of the jaw

Abstract

Intraosseous vascular malformation (IVM) is a relatively rare pathological condition that may pose significant risks, such as excessive bleeding, during surgical procedures. We present a case of an 18-year-old female patient with firm swelling in the left maxilla. A bony expansion over the left half of the maxilla with preservation of the outer cortex and a ground glass appearance on CT images initially revealed a possibility of fibrous dysplasia. However, a tentative diagnosis of IVM was made based on the vascular nature of the lesion as well as the patient's surgical history and additional imaging findings. IVM should be included in the differential diagnosis of an expansile bony lesion with trabecular alteration. Through the literature review, it was found that imaging findings, such as a neurovascular canal widening on CT images and a hyperintense signal on T1 weighted MR images, might be helpful in differentiating IVM from other pathologies.

A rapidly growing expansile lesion of the maxillary sinus in a child

“…12 Intraosseous vascular malformations account for less than 1% of all bony tumors and affect the vertebral column and calvarium more frequently. 14 In the maxillofacial region, vascular malformations most commonly occur in soft tissue versus the facial bones. 15,16 The radiographic appearance of these lesions is variable, but they are usually seen as well-defined, multilocular, radiolucent defects.…”

“…2 Case reports, such as those by Kim et al and Kacker et al, have described cases of intrabony vascular malformations affecting the maxilla of young patients. 14,16 An important diagnostic feature of these lesions is that a bruit or pulsation may be detected clinically. This was not the case in our patient.…”

Loss-of-Function Mutations in ELMO2 Cause Intraosseous Vascular Malformation by Impeding RAC1 Signaling

“…The facial appearance of individuals with VMOS and their CT images resemble cherubism, as mandibular bone is replaced by excessive amounts of fibrous tissue. 10,12,13 Pathological findings are critical for differential diagnosis, because there are no pathognomonic radiographic findings for VMOSs. 11,13,14 Various terms have been used to describe malformations similar to VMOSs, including intraosseous cavernous hemangioma, extraspinal osseous hemangioma, central hemangioma, cavernous angiomata of skull, and cystic angiomatosis.…”

“…10,12,13 Pathological findings are critical for differential diagnosis, because there are no pathognomonic radiographic findings for VMOSs. 11,13,14 Various terms have been used to describe malformations similar to VMOSs, including intraosseous cavernous hemangioma, extraspinal osseous hemangioma, central hemangioma, cavernous angiomata of skull, and cystic angiomatosis. 12,[14][15][16] Recently, the nomenclature of vascular bone lesions was updated and earlier cases were re-classified according to the new ISSVA classification.…”