Glomeruloid haemangioma: a possible consequence of elevated VEGF in POEMS and Erdheim-Chester disease.
Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective monocentric study of histologically-confirmed GH. Clinical, biological and histological data of the patients is presented. In addition to the two presented cases, 11 GH histologically-confirmed cases were retrospectively identified. Six patients were female (46.2%; 95 CI: 12-64.9) and median age was 54 years (31-85). For 11 patients (84.6%; 95 CI: 65-104.2), a diagnosis of POEMS syndrome was retained, one patient had autoimmune hepatitis, and another had ECD. GH was localised to the trunk in 10 cases (76.9%; 95 CI: 54-99) and the legs in the other three. The median number of haemangiomas in the cohort was three (SD: 3.08). Median level of VEGF was 1,490 (610-12,000) ng/mL. All immunohistochemical staining for human herpesvirus 8 (HHV-8) was negative. Of the 13 cases of GH, of which two were not clear-cut POEMS syndrome, we report the first case of GH associated with ECD. In this cohort, all patients had high serum levels of VEGF but no in situ HHV-8 latent infection. We hypothesise that GH might be linked to a high level of VEGF in these two rare diseases.
['Legendre|Paul|P|', 'Norkowski|Emma|E|', 'Le Pelletier|François|F|', 'Miyara|Makoto|M|', 'Choquet|Sylvain|S|', 'Haroche|Julien|J|', 'Brocheriou|Isabelle|I|', 'Barete|Stéphane|S|']
D000328:Adult D000368:Aged D000369:Aged, 80 and over D031249:Erdheim-Chester Disease D005260:Female D006391:Hemangioma D006801:Humans D008297:Male D008875:Middle Aged D016878:POEMS Syndrome D012189:Retrospective Studies D012878:Skin Neoplasms D047368:Tumor Burden D042461:Vascular Endothelial Growth Factor A
glomeruloid haemangioma Erdheim-Chester disease POEMS syndrome hairy kidney serum VEGF