Dabska Tumor Developing Within a Preexisting Vascular Malformation

Abstract

Malignant endovascular papillary angioendothelioma, first described by Dabska in 1969, is a rare vascular tumor that primarily affects children and is characterized by papillary proliferations of endothelial cells into vessel lumens. We report a case of this rare neoplasm in a boy with angiomatosis who developed Kasabach-Merritt syndrome. The tumor evolved as an ulcerated lesion superficially within a previous vascular malformation on his buttock. A review of the literature is presented.

Endovascular papillary angioendothelioma (Dabska tumor): underrecognized malignant tumor in childhood

“…Of note, no other primary sources were discovered in these instances, nor was lymph node involvement present. In addition, because more cases are being reported and tumors are being classified as being EPAs, some are thought to develop from preexisting vascular lesions [7,8]. In Dabska's original article, 3 of the 6 patients had congenital lesions diagnosed, whereas the remainder had clinically notable lesions that developed later.…”

The Dąbska Tumor: A Thirty-Year Retrospect

“…It may fluctuate in size and tends to be associated with degeneration of an underlying vascular tumor. DT has been described as a focal degeneration within a deep cavernous hemangioma of leg soft tissue in an 8-year-old girl [7], within an angiomatous vascular malformation on the buttocks of a boy who developed the Kasabach-Merritt syndrome [4], and as part of a subcutaneous cavernous hemangioma fixed to underlying fascia on the forearm of a 6-year-old boy [5]. One or more of these ‘hemangiomas’ may actually have been lymphangiomas [8].…”

“…The tumor cells express immunoreactivity for factor-VIII-related antigen, Ulex europaeus I lectin, vimentin, blood group isoantigens and C 2.1 antibody, but not for S-100 protein, cytokeratins, epithelial membrane antigen, Leu-M1, HLA-DR, α 1 -antichymotrypsin and leukocyte common antigen [4, 6, 7, 8, 19]. The factor-VIII-related antigen and U. europaeus I lectin immunoreactivity and other findings, even in the absence of HLA-DR expression, suggest that the neoplastic cells may differentiate towards ‘high’ endothelial cells, ones with surface receptors that serve as ligands in the selective homing of lymphocytes in lymphoid organs [16].…”

“…The Dąbska tumor (DT), a rare angiosarcoma also known as malignant endovascular papillary angioendothelioma of childhood, is so unusual that only about 30 patients have been described [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11]. Enzinger and Weiss [10]in 1996 called it ‘a form of low-grade angiosarcoma occurring in skin or subcutis of infants or young children, although we have seen rare cases in adults’.…”

Congenital Occipital Encephalocele with Dabska Tumor: Report of an Unusual Case

“…These tumors appear to be closely related to retiform hemangioendotheliomas. There have been cases in the literature which have documented Dabska tumors arising in preexisting benign vascular tumors or malformations [8,9]. Rare cases of Dabska tumor have been seen in the spleen and other deep locations [10].…”