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Soft Tissue Pathology 

Pathology of Malignant Endovascular Papillary Angioendothelioma (Dabska Tumour)

 Dr Sampurna Roy MD           

 

                                                                                                                      

 

 

Malignant endovascular papillary angioendothelioma, a rare but distinctive vascular tumour of childhood, was first described in 1969 by Maria Dabska. Malignant endovascular 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.

Microscopic features:

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 hemangiomaIntravascular papillary endothelial hyperplasia (Masson's tumour) ; Reactive angioendotheliomatosis; Angiosarcoma.

 

Further reading:

Unusual multifocal intraosseous papillary intralymphatic angioendothelioma (Dabska tumor) of facial bones: a case report and review of literature.

Strong ERG Positivity in Papillary Intralymphatic Angioendothelioma of the Testis of a 24-Year-Old Male: A Case Report.

Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours.

Endovascular papillary angioendothelioma (Dabska tumor) of the tongue: report of a case.

The Dabska tumor: a thirty-year retrospect.

Papillary intralymphatic angioendothelioma (PILA): a report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels.

Endovascular papillary angioendothelioma (Dabska tumor).

Dabska tumor developing within a preexisting vascular malformation.

Malignant endovascular papillary angioendothelioma (Dabska tumor). A case report and review of the literature.

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.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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