Papillary intralymphatic angioendotelioma: case report

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Papillary intra-lymphatic angioendothelioma (PILA) is a rare vascular tumor which was first characterized in 1969 by María Dabska, who described in her series of 6 cases a low-grade angiosarcoma, usually developed in childhood and that had distinctive histological characteristics. Since then about 3...

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Autores principales: Defazio , ND, Rivas , DA
Formato: Artículo revista
Lenguaje:Español
Publicado: Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019
Materias:
angioendothelioma
tumors
skin
neoplasms
Acceso en línea:https://revistas.unc.edu.ar/index.php/med/article/view/25658
Aporte de:
Revistas de la UNC de Universidad Nacional de Córdoba
id I10-R10article-25658
record_format ojs
institution Universidad Nacional de Córdoba
institution_str I-10
repository_str R-10
container_title_str Revistas de la UNC
language Español
format Artículo revista
topic angioendothelioma
tumors
skin
neoplasms
spellingShingle angioendothelioma
tumors
skin
neoplasms
Defazio , ND
Rivas , DA
Papillary intralymphatic angioendotelioma: case report
topic_facet angioendothelioma
tumors
skin
neoplasms
author Defazio , ND
Rivas , DA
author_facet Defazio , ND
Rivas , DA
author_sort Defazio , ND
title Papillary intralymphatic angioendotelioma: case report
title_short Papillary intralymphatic angioendotelioma: case report
title_full Papillary intralymphatic angioendotelioma: case report
title_fullStr Papillary intralymphatic angioendotelioma: case report
title_full_unstemmed Papillary intralymphatic angioendotelioma: case report
title_sort papillary intralymphatic angioendotelioma: case report
description Papillary intra-lymphatic angioendothelioma (PILA) is a rare vascular tumor which was first characterized in 1969 by María Dabska, who described in her series of 6 cases a low-grade angiosarcoma, usually developed in childhood and that had distinctive histological characteristics. Since then about 30 cases have been reported, mostly in the skin and subcutis, but also in deep organs such as spleen, bone and testis. The objective of our work was to communicate a case of this very rare vascular neoplasm, review its clinical aspects, its gross and microscopic appearance and establish its biological behavior.  Our case corresponded to a 9 year old girl with a lower lip injury. A wedge-shaped fragment partially covered by 5 x 2.5 cm semi-mucosa was received. In one sector, a lesion of warty, papillary appearance of approximately 3 x 1 cm was observed. Microscopy showed a skin proliferation of vessels covered by cuboid cells, enlarged in size. The endothelial cells had a "hobnail" appearance with nuclei protruding towards the light. They were arranged constituting papillae and in other sectors forming glomeruloid structures. These findings were compatible with a papillary intra-lymphatic angioendothelioma. In immunohistochemistry it was positive for CD34. PILA is a rare vascular neoplasm with a high rate of local recurrences but with infrequent metastases. Differential diagnoses must be taken into account for which immunohistochemistry techniques can be used
publisher Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
publishDate 2019
url https://revistas.unc.edu.ar/index.php/med/article/view/25658
work_keys_str_mv AT defaziond papillaryintralymphaticangioendoteliomacasereport
AT rivasda papillaryintralymphaticangioendoteliomacasereport
AT defaziond angioendoteliomaintralinfaticopapilarreportedeuncaso
AT rivasda angioendoteliomaintralinfaticopapilarreportedeuncaso
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