Vascular tumors of intermediate malignancy encompass a broad range of histologic entities. They are characterized clinically by having a high risk of local recurrence and limited risk of regional or distant metastasis. Vascular tumors of intermediate malignancy frequently present in the skin and often cause diagnostic diffi culty. This article reviews the clinical and histologic features of kap...

Kaposiform hemangioendothelioma is a rare vascular tumor and locally aggressive endothelial-derived spindle cell neoplasm, which occurs almost exclusively in infants and adolescents. Radiologically, hemangioendothelioma, including Kaposiform hemangioendothelioma, is seen as a highly vascularized well-enhancing tumor, but no characteristic findings differentiate Kaposiform hemangioendothelioma f...

Infantile hemangioendothelioma is a severe disease with a high mortality. It is characterized by multiple hemangioma affecting the skin and visceral organs. We report that high doses of methylprednisolone pulse therapy improved symptoms and signs of infantile hemangioendothelioma in a male neonate, and completely resolved the hepatic and cutaneous hemangioendothelioma on follow up.

Kaposiform hemangioendothelioma, a rare vascular pediatric tumor often associated with Kasabach-Merritt phenomenon, is characterized by severe thrombocytopenia and consumptive coagulopathy. Kaposiform hemangioendothelioma is a severe disease and may progress quickly, resulting in a high mortality. However, standard treatment regimens for Kasabach-Merritt phenomenon have not yet been established...

Bartonella henselae and B. koehlerae bacteremia was documented in two epithelioid hemangioendothelioma patients and B. koehlerae bacteremia in an asymptomatic partner of one of the patients. Considering the biology and clinically variable natural history of epithelioid hemangioendothelioma, these results suggest that chronic Bartonella infection could have a role in the development of this vasc...

Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed...

BACKGROUND Pseudomyogenic hemangioendothelioma is a rare soft tissue tumor usually found in young adults, predominantly males. Fibrous dysplasia is a common benign bone tumor, which accounts for 5~7 % of all the primary benign bone tumors. However, pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia is extremely rare. To the best of our knowledge, this is the first case of pseudo...

Hepatic hemangioendothelioma is rare. We report on hepatic hemangioendotheliomas in 2 young infants, with initial manifestations of respiratory distress and congestive heart failure. Serum alpha-fetoprotein (alphaFP) level was as high as 26,343 microg/l at 14 days old in 1 case, but was only 18 microg/l in the other case. The 2 patients were treated with prednisolone and hepatic artery ligation...

1. Calonje E, Fletcher CD, Wilson-Jones E, et al. Retiform hemangioendothelioma: a distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25. 2. Hirsh AZ, Yan W, Wei L, et al. Unresectable retiform hemangioendothelioma treated with external beam radiation therapy and chemotherapy: a case report and review of the literature. Sarcoma 2010;2010:...

BACKGROUND Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignant potential and only 10 cases have been described in the literature so far. METHODS AND RESULTS We report a case of nodal and extranodal polymorphous hemangioendothelioma and review the literature. A 66-year-old man with an unremarkable past medical history was admitted for a left submandibular mass. T...