[91] Papillary Hemangioma and Glomeruloid Hemangioma Are Distinct Clinicopathological Entities

AJ Suurmeijer. University Medical Center, Groningen, Netherlands

Background: Papillary hemangioma was recently defined as a morphologically distinct and benign cutaneous hemangioma showing a predominantly intravascular capillary proliferation within dilated thin-walled dermal blood vessels. Glomeruloid hemangioma is a very rare intravascular capillary hemangioma, that structurally resembles the renal glomerulus, and is regarded to be specific for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin lesions) syndrome and/or multicentric Castleman's disease. Given their curiosity and mimicry, the differential diagnosis can be difficult and troubling.
Design: Clinicopathologic features of eleven cases of papillary hemangioma recently published were compared with those of five glomeruloid hemangiomas collected through communication with authors who had previously reported on the entity. Immunostaining for CD34, podoplanin, smooth muscle actin, and collagen IV was performed on five papillary hemangiomas and three glomeruloid hemangiomas.
Results: Morphologically, papillary hemangiomas showed a predominant intravascular growth within one or more ectatic dermal blood vessels. Papillary formations contained pericytes and stromal cells, but few capillaries. Luminal surface endothelial cells contained numerous eosinophilic hyaline globules. Glomeruloid hemangiomas also were dermal intravascular lesions, albeit with a glomeruloid appearance due to the presence of numerous capillary loops. Glomeruloid hemangiomas also contained hyaline globules, but less than found in papillary hemangiomas. Immunostaining for CD34, smooth muscle actin, and, in particular, collagen IV highlighted discriminating features. Thin basement membranes and glomeruloid architecture were typical of glomeruloid hemangioma, whereas papillae with thick mantles of basement membrane-like matrix enveloping stromal cells and pericytes were prominent in papillary hemangioma. Both entities were negative for podoplanin. Clinically, glomeruloid hemangioma often presenting in a spectrum of multiple cutaneous vascular lesions including cherry hemangiomas - was a hallmark of POEMS syndrome and/or multicentric Castleman's disease, whereas papillary hemangioma presented as an innocent solitary cutaneous hemangioma in the head and neck region of otherwise healthy individuals.
Conclusions: Distinct collagen IV immunostaining patterns in papillary hemangioma and glomeruloid hemangioma should enable pathologists to discriminate beween these lesions, which is clinically relevant, because glomeruloid hemangioma may be part of POEMS syndrome.
Category: Bone & Soft Tissue

Tuesday, March 10, 2009 9:30 AM

Poster Session III # 3, Tuesday Morning