Cutaneous symplastic hemangioma: A series of four cases

Symplastic hemangiomas (SH) are benign vascular lesions that show atypia in vascular smooth muscle and interstitial cells with sparing of endothelial cells. We present four cases of this rare tumor. The patients (two males; two females) ranged in age from 57 to 83 years (median 74); lesions were located on the leg (n = 3) and back (n = 1), and ranged from 6 to 8 mm. SH were well-circumscribed and dermal-based, often with an epidermal collarette (3/4). They were characterized by the presence of variably atypical, hyperchromatic/pleomorphic epithelioid to spindled cells within vascular walls (3/4) and/or perivascular stroma (4/4). Atypical multinucleated cells were present in three of four cases. The overall mitotic rate was low (0-2 mitotic figures per 10 HPFs; mean 0.75 per 10 HPFs), but atypical mitotic figures were seen in two of four cases. Atypical cells were negative for SMA (0/2), desmin (0/2), AE1/3 (0/2), and CAM5.2 (0/1). ERG, CD31, and CD34 stains were positive in endothelial cells but negative in atypical cells (4/4). Lesional cells and vessels were negative for podoplanin (3/3). Symplastic hemangioma is a benign tumor with bizarre atypia that may be mistaken for malignancy. We present four cases of this rare entity to increase awareness of this tumor and discuss the differential diagnosis.