Papillary intralymphatic angioendotelioma: case report

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 Córdoba. 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:
Revista de la Facultad de Ciencias Médicas de Córdoba de Universidad Nacional de Córdoba
id I10-R327-article-25658
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institution Universidad Nacional de Córdoba
institution_str I-10
repository_str R-327
container_title_str Revista de la Facultad de Ciencias Médicas de Córdoba
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 Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
publishDate 2019
url https://revistas.unc.edu.ar/index.php/med/article/view/25658
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AT rivasda papillaryintralymphaticangioendoteliomacasereport
AT defaziond angioendoteliomaintralinfaticopapilarreportedeuncaso
AT rivasda angioendoteliomaintralinfaticopapilarreportedeuncaso
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spelling I10-R327-article-256582024-08-27T18:26:02Z Papillary intralymphatic angioendotelioma: case report Angioendotelioma intralinfatico papilar: reporte de un caso Defazio , ND Rivas , DA angioendothelioma tumors skin neoplasms 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 El angioendotelioma intralinfático papilar ( AIP ) es un tumor vascular infrecuente, que fué caracterizado por primera vez en 1969 por María Dabska, quien describió, en su serie de  6 casos, un angiosarcoma de bajo grado, habitualmente desarrollado en la infancia y que poseía características histológicas distintivas. Desde entonces unos 30 casos han sido reportados, la mayoría en la piel y TCS, pero también en órganos profundos como bazo, hueso y testículo.  El objetivo de nuestro trabajo fue comunicar un caso de esta neoplasia vascular muy poco frecuente, repasar su clínica, su apariencia macro y microscópica, y establecer su comportamiento biológico.                          PRESENTACION DEL CASO: Nuestro caso correspondió a una niña de 9 años con una lesión en labio inferior. Se recibió un fragmento en forma de cuña revestido parcialmente por semi-mucosa de 5 x 2.5 cm. En un sector se observó una lesión de apariencia verrugosa, papilar, de aproximadamente 3 x 1 cm. En la microscopia se observó una proliferación cutánea de vasos revestidos por células cuboideas, aumentadas en tamaño.  Las células endoteliales tenían una apariencia “en tachuela”, con núcleos protruidos hacia la luz. Se disponían constituyendo papilas y en otros sectores conformando estructuras glomeruloides. Estos hallazgos resultaron compatibles con un angioendotelioma intralinfático papilar. En la inmunohistoquímica resultó positivo para CD34. El AIP es una neoplasia vascular rara, con alta tasa de recidivas locales, pero con metástasis infrecuentes. Se deben tener en cuenta diagnósticos diferenciles, para los cuales se puede recurrir a técnicas de inmunohistoquímica. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019-10-10 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/25658 Revista de la Facultad de Ciencias Médicas de Córdoba.; 2019: Suplemento JIC XX Revista de la Facultad de Ciencias Médicas de Córdoba; 2019: Suplemento JIC XX Revista da Faculdade de Ciências Médicas de Córdoba; 2019: Suplemento JIC XX 1853-0605 0014-6722 10.31053/1853.0605.v76.nSuplemento spa https://revistas.unc.edu.ar/index.php/med/article/view/25658/27374 Derechos de autor 2019 Universidad Nacional de Córdoba https://creativecommons.org/licenses/by-nc/4.0

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