Abstract

Papillary hemangioma (PH) is a rare benign vascular neoplasm with less than 20 documented cases that tends to occur on the head and neck region of otherwise healthy individuals. First described in 2007, PHs share significant histopathologic resemblance with glomeruloid hemangiomas (GHs), which are strongly associated with multicentric Castleman’s disease and POEMS (polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal protein, skin changes) syndrome. Despite their morphologic similarities, the two entities have distinguishable features on H&E. We present a case of PH in a 69-year-old man with history of diabetes, and hypercholesterolemia who presented for a 3-year history of persistent raised lesion on his left upper back. Skin examination revealed well-demarcated, dome-shaped, soft, reddish-purple nodule overlying the left scapular region. Punch biopsy revealed a mid-deep dermal and superficial subcutaneous nodular vascular proliferation comprised of papillary fronds lined by hobnail endothelial cells and scattered deeply eosinophilic intracytoplasmic hyaline globules. A diagnosis of PH was favored. Out of an abundance of caution, the patient underwent a complete blood count, serum and urine electrophoresis which did not uncover any abnormalities concerning for POEMS syndrome or Castleman’s disease. Our case illustrates the architectural pattern that distinguishes PHs from GHs: the former with papillary proliferations lined by plump endothelial cells, often containing abundant basement membrane–like collagen material whereas the latter form glomeruloid conglomerations of capillary endothelial cells lined by pericytes and separated from other capillaries by polygonal stromal cells. Histologic delineation between PH and GH is clinically meaningful as the latter typically necessitates workup for underlying syndromic associations.