Abstract:

Papillary hemangioma (PH) is a recently described vascular tumor frequently involving the skin of the head and neck region. Histopathologically, it is characterized by a bland endothelial proliferation arranged in a papillary configuration, bearing resemblance to the glomeruloid hemangioma, as seen in the context of POEMS syndrome. Fletcher and colleagues reported a median tumor size of 1.2 cm (0.3-2 cm). Here, we report a case of a PH with an unusually large size. A 38-year-old male presented with an enlarging scalp lesion, first noticed by his hairdresser, 3 years ago. Physical examination revealed an irregular, violaceous to bluish black, papulonodular lesion on his right scalp, measuring 10 cm. Surgical excision with skin grafting was performed. Microscopic examination revealed a sporadic intravascular growth of endothelial cells with papillary fronds anchored to the vessel wall. There were papillary cores containing capillaries lined by uniform, cytologically bland endothelial cells. Eosinophilic intracytoplasmic hyaline globules were present. Immunohistochemical stains for CD31 and ERG were positive in the endothelial cells whereas podoplanin was negative. SMA highlighted pericytes underneath endothelial cells. Significant nuclear atypia, aberrant mitotic activity, and infiltrative growth were not observed. Clinical signs of POEMS syndrome were absent. A diagnosis of PH was rendered. The margins of the excision were narrowly negative for tumor. At 6-month follow-up, no signs of recurrence were identified. We present this case to add to the limited literature on this rare tumor, highlight the histopathologic differences between PH and its mimickers, and emphasize the alarming size PH can manifest.

Financial Disclosure:
No current or relevant financial relationships exist.

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Aneeza Irfan, MBBS, MD