Kaposiform hemangioendothelioma: a clinicopathological analysis of 29 cases / 临床与实验病理学杂志

ABSTRACT

Purpose To explore the clinicopathologic features, diagnosis and differential diagnosis of Kaposiform hemangioendothelioma. Methods To reported the histopathological and immunohistochemical features in 29 cases of Kaposiform hemangioendothelioma, combined with the clinical data and the literature review. Results Of the 29 patients, 15 were male and 14 were female, almost all patients were no more than 1 year old, except one patient aged 25 years.28 cases occurred in superficial skin, including 15 cases in limbs, 5 cases in head and neck, 4 cases in trunk, 1 case in armpit, 3 cases were multiple sites, and 1 case in pelvic cavity. The clinical manifestations were limited or diffuse skin painless plaque, and 16 cases with Kabasach-Merritt phenomenon. Microscopically, the tumor was composed of proliferating small blood vessels and spindle cells, with irregular nodular or lobulated distribution, and invasive growth. Spindle cells were arranged in bundles, with epithelioid cells and spindle cells arranged in nests, and glomerulus-like structures, and the latter with crescent shaped vascular fissures. Immunohistochemically, the tumor cells were strongly positive for VEGFR-3, CD31, CD34 and ERG. Partial tumor cells were positive for SMA and D2-40, but negative for CK, Glut-1 and HHV8. Conclusion Kaposiform hemangioendothelioma is a rare, intermediate, locally aggressive angiogenic tumor, mainly occurring in infants and young adults. The diagnosis relies on its unique morphology and immunophenotype, and it should be differentiated from tufted angioma, infantile hemangioma, congenital hemangioma, spindle cell hemangioma, verrucous venous malformation and Kaposi sarcoma. Extended resection is the best way to treat Kaposiform hemangioendothelioma.