A Rare Case of Intraosseous Papillary Hemangioma of the Head and Neck

Abstract

Objectives Papillary hemangioma (PH) is a novel variant of intravascular capillary hemangioma. It is more common in adults and has a male predominance. Most tumors reported so far are solitary and cutaneous. Here we present a rare case of an intraosseous PH involving the frontal bone. Methods Brain imaging in a 69-year-old male with a slowly enlarging swelling on the right frontal area following an accidental fall demonstrated a 4.5 cm x 1.7 cm x 4.2 cm mass originating from the right frontal bone, with a tiny defect on the orbital roof. A malignant process was favored, and the mass was removed. Results Histopathology revealed a vascular lesion showing intraosseous distribution with foci of extension into the fibrous connective tissue. There were areas of plump endothelial cells with intracytoplasmic hyaline globules arranged in papillary configuration. The lesional cells were immunoreactive with CD34 and vimentin. AE1/AE3, EMA, PR, D2-40, inhibin and S100 stains were negative. Ki-67 was low. Conclusion This is the first intraosseous and second non-cutaneous PH. Clinically it differs from other cases by the presence of trauma as a preceding event. Since its prognosis is unknown such patients should be monitored for recurrence or malignant transformation.

Papillary Hemangioma Harbors Somatic GNA11 and GNAQ Mutations

“…1 The dermal lesion is typically composed of dilated, thin-walled channels with papillary clusters of mainly capillary-sized vessels and endothelial cytoplasmic hyaline globules. Since their initial cohort of 11 patients, a limited number of case reports have identified few additional cases, [2][3][4] presently, molecular alterations in PH have not been identified. These lesions follow a benign clinical course and treatment is complete excision; however, 1 patient in the original study presented 10 years after initial diagnosis with a recurrent lesion.…”