Retiform Hemangioendotheliomas Usually do not Express D2-40 and VEGFR-3

Department of Pathology, Medical Center, Medical Center Boulevard, Wake Forest University Baptist, Winston-Salem, NC, USA.
The American Journal of dermatopathology (Impact Factor: 1.39). 03/2008; 30(1):31-3. DOI: 10.1097/DAD.0b013e31815ea7c5
Source: PubMed


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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    • "The “plump and hobnail-like hemangioendothelioma” can also be seen in cases of Dabska tumor.[7] Of note, RH may stain partially positive for D2-40, but it does not usually express VEGR-3.[10] Kaposiform hemangioendothelioma can mimic RH, as it may involve the skin during infancy and early childhood, despite typically presenting as a retroperitoneal tumor with abdominal mass, jaundice, or ascites. "
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    ABSTRACT: Retiform hemangioendothelioma (RH) is a distinct entity in the spectrum of vascular tumors with a high local recurrence rate. It is considered a low-grade, well-differentiated cutaneous angiosarcoma with low metastatic potential. We report here for the first time a case of medial canthus recurrent RH. It may be helpful in our practice to include RH as a differential diagnosis of eyelid lesions. It is noteworthy that the progressive course and recurrence tendency of RH might be misdiagnosed as angiosarcoma or basal cell carcinoma (BCC), if not expected and carefully evaluated by the pathologist.
    No preview · Article · Apr 2014 · Indian Journal of Ophthalmology
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    • "In our case, D2-40 antibody reacted only in normal vascular endothelium, representing the internal control, and failed to demonstrate a convincing lymphatic differentiation, findings suggestive of the diagnosis of RH [8,9]. "
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    ABSTRACT: Retiform hemangioendothelioma (RH) is an uncommon vascular neoplasm of borderline malignancy that clinically develops as a solitary, gradually enlarging exophytic mass, nodule or plaque, most often on the lower limbs, upper limbs and trunk. Clinical recognition of RH is troublesome because of its non-specific appearance, with differential diagnosis comprising a variety of benign and malignant tumors clinically presenting as reddish nodules. In this article we describe the clinical, dermoscopic and histopathologic findings in a case of RH developing on the flank of a 26-year-old woman, and discuss the possible role of dermoscopy in facilitating the clinical recognition of this rare tumor.
    Full-text · Article · Oct 2013
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    • "In summary, based on the staining patterns, it has been suggested that RH is a vascular entity which usually does not have lymphatic differentiation [13]. Other vascular tumors that morphologically can present similarly to RH and need to be differentiated from RH include Dabska's tumor, lymphoma, dermatofibrosarcoma protuberans, hemangioma, bacillary angiomatosis, cutaneous metastases, blue-rubber bleb nevus syndrome, Kaposi's sarcoma, targetoid hemosiderotic hemangioma, hobnail hemangioma, epitheloid hemangioendothelioma, and polymorphous hemangioendothelioma of lymph node [1] [2] [7] [9]. "
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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.
    Full-text · Article · Sep 2010 · Sarcoma
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