Pathology of Malignant Endovascular Papillary Angioendothelioma (Dabska Tumour)
papillary angioendothelioma, a rare but distinctive vascular tumour of
childhood, was first described in 1969 by Maria Dabska.
papillary angioendothelioma of the skin in childhood.
Clinicopathologic study of 6 cases.
It was termed malignant because of its mitotic activity, areas of necrosis, and demonstrated ability to metastasize to regional lymph nodes.
Based on its good long-term prognosis and variable histologic features, this lesion is better classified as a borderline vascular tumour.
According to some authors endovascular papillary angioendothelioma may not be a distinct entity and may well include a heterogeneous group of lesions.
Age: Mostly occurs in infants and children. Cases have also been reported in adults.
Site: Slow growing pink or bluish-black nodule located in the skin or soft tissue . There is slight predominance in the head and neck region and extremities.
Image Link: (dermpath.de) (Courtesy of Thomas Mentzel, MD)
Presence of anastomosing vascular channels.
Prominent endothelial intraluminal papillary tufts with hyaline cores surrounded by lymphocytes.
The vascular spaces are lined ny monomorphic hobnail endothelial cells.
In some areas glomeruloid structures are identified.
[ The three cell types originally described are as follows:
i) Lymphocyte-like cells
ii) Large atypical cells sometimes showing hobnail appearance
iii) Flat endothelial cells ]
Papillary endothelial proliferation with a central hyaline core is one of the most characteristic features, however, this type of proliferation has been observed in other vascular tumours, such as angiosarcoma, retiform hemangioendothelioma and glomeruloid hemangioma.
Immunohistochemistry: Tumour cells are positive for vimentin, von Willebrand factor, CD31, and focally for CD34 and are negative for keratins, epithelial membrane antigen, S-100 protein, and desmin.
Differential diagnosis: Retiform hemangioendothelioma ; Hobnail hemangioma; Glomeruloid hemangioma; Intravascular papillary endothelial hyperplasia (Masson's tumour) ; Reactive angioendotheliomatosis; Angiosarcoma.
angioendothelioma (PILA): a report of twelve cases of a distinctive
vascular tumor with phenotypic features of lymphatic vessels.
Vascular endothelial growth factor receptor-3 (VEGFR-3): a marker of vascular tumors with presumed lymphatic differentiation, including Kaposi's sarcoma, kaposiform and Dabska-type hemangioendotheliomas, and a subset of angiosarcomas.Mod Pathol. 2000 ;13(2):180-5.
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