Massive craniofacial cystic angiomatosis and extra-skeletal angiomatosis: A unique case report and brief review of literature

Vascular Floor of Mouth Mass with Unanticipated Intracranial, Orbital, and Vertebral Associated Involvements

“…CA is a rare, idiopathic proliferation of vascular tissue typically in the medullary spaces of long bones and axial skeleton [2,3]. Its oft-lytic lesions are multilocular, well-defined, cystic entities with visceral spread commonly noted in the spleen, liver, kidney, pleura, and peritoneal membrane [1][2][3][4]. Polyostotic presentations may mimic metastatic disease [1,5].…”

“…The distended, thin-walled vessels on histology likely represent lymphangiomatous proliferations marked by vascular endothelial growth factor (VEGF) and podoplanin [1,2]. CD31 (or PECAM-1) and factor VIII antigen are other notable markers of CA, indicating a hematopoietic lineage [3,6].…”

“…Literature on CA is sparse, with several studies suggesting a young male predominance [1,[4][5][6] while others purport a preference for middle-aged females [2,3]. A survey of the MEDLINE database showed approximately 50 known cases in the English literature [1].…”

“…A survey of the MEDLINE database showed approximately 50 known cases in the English literature [1]. Though calvarial presentations are documented, maxillary and mandibular variants have each been reported in fewer than 5 cases as of 2016 [3,5]. The mean age of presentation within the documented cases was 22.8 with a slight male predominance [1].…”

“…Three patients demonstrated mandibular lesions and another a maxillary lesion upon radiography. One individual complained of rhinorrea also indicating naso-maxillary involvement [3]. The first known case of posterior fossa and cerebellar compression was also published in 2015, but this presentation did not involve the facial bones [1,3,5].…”