A Case of Oral Glomeruloid Hemangioma Without Systemic Conditions

Glomeruloid hemangioma is a rare variant of hemangioma that is accompanied by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome and, rarely, by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome. This report presents the case of a 78-year-old male who presented with a hemorrhagic nodule on the tongue without any other systemic diseases. Microscopically, the lesion was a lobular proliferation extending from the lamina propria to muscular tissue. Some intravascular nodules with irregular vascular lumens closely resembled renal glomeruli. Each nodule consisted of plump endothelial and stromal cells that partially showed vacuolated cytoplasm containing eosinophilic and periodic acid-Schiff (PAS)-positive globules. Immunohistochemically, IgG-positive deposition was noted within CD31-positive cells. Many plump stromal cells were positive for CD31, CD146, nestin, and type IV collagen but not α-smooth muscle actin (αSMA). These results reflect the proliferation of immature endothelial cells and pericytes, which might characterize this unique lesion. Microscopically, this case revealed glomeruloid hemangioma without systemic conditions related to POEMS, and composed of an intravascular proliferation of immature endothelial and pericytic stromal cells.