Retiform hemangioendotheliomas usually do not express D2-40 and VEGFR-3
ABSTRACT Retiform hemangioendothelioma (RH) is a rare vascular neoplasm most often occurring in the limbs of middle-aged females. This entity is characterized by infiltrative vascular spaces arranged in a pattern similar to the rete testis. RH differs from angiosarcoma by lacking cytologic atypia and high mitotic rates. This neoplasm frequently recurs but rarely metastasizes. RH tumor cells react with vascular endothelial markers CD31, CD34, and factor VIII-related antigen. A review of the English literature provides only one attempt at staining RH with D2-40, a marker of endothelium of lymphatic vessels, which was negative, and one reported staining of RH with lymphatic endothelial marker VEGFR-3, which was positive. The etiology of RH is unknown. RH has previously been considered closely related to Dabska tumors, which are positive for lymphatic endothelial marker D2-40. We stained 4 RHs with mouse monoclonal antibodies against D2-40 and CD31 and 3 of the 4 RHs with vascular endothelial growth factor receptor 3 (VEGFR-3), to further evaluate whether RH had lymphatic differentiation, in addition to vascular differentiation. Three of the 4 RH biopsies failed to demonstrate D2-40, none expressed VEGFR-3, whereas CD31 was strongly positive, suggesting that RH is a vascular entity which usually does not have lymphatic differentiation, but may rarely express D2-40.
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ABSTRACT: Retiform hemangioendothelioma (RH) is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy. Treatment of RH is controversial. We present a case of a 44-year-old Asian male presenting with an unresectable RH of the pelvis. The patient was treated with concurrent low-dose Cisplatin and External beam Radiation (4140cGy in 180cGy per fraction). This is the first report of a clinical complete response and a long-term local control of this rare tumor. This has significant clinical implication, since it gives the first evidence of treatment of this rare tumor using concurrent low-dose chemotherapy and radiation.Sarcoma 09/2010; 2010. DOI:10.1155/2010/756246
- The American Journal of dermatopathology 07/2008; 30(3):302-4. DOI:10.1097/DAD.0b013e31816ddb45 · 1.43 Impact Factor
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ABSTRACT: An 8-year-old female presented with a recurrent hobnail (Dabska-retiform) hemangioendothelioma of her right foot. After initial tumor excision and subsequent recurrence, the patient underwent a successful tumor resection and forefoot reconstruction using a fillet of second digit flap. In an attempt to create a plantigrade foot, while sufficiently removing this rare pedal tumor, the authors employed the use of a fillet of second digit flap. Specific surgical techniques and the histopathological assessment of this rare vascular neoplasm are discussed. Level of Clinical Evidence: 4.The Journal of foot and ankle surgery: official publication of the American College of Foot and Ankle Surgeons 09/2008; 47(5):487-93. DOI:10.1053/j.jfas.2008.06.004 · 0.98 Impact Factor