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Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors

Angiomyolipoma and PEComa are rare tumors descending from perivascular epithelial cells (PECs), with distinctive IHC, morphological, and ultrastructural features. The kidney is the most frequent site of origin, but not the only one; however, adrenal gland angiomyolipomas are extremely rare. We descr...

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Detalles Bibliográficos
Autores principales: Kwazneski II, Douglas, Merrill, Megan, Young, Jessica, Sell, Harry
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4779821/
https://www.ncbi.nlm.nih.gov/pubmed/26998374
http://dx.doi.org/10.1155/2016/5204092
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author Kwazneski II, Douglas
Merrill, Megan
Young, Jessica
Sell, Harry
author_facet Kwazneski II, Douglas
Merrill, Megan
Young, Jessica
Sell, Harry
author_sort Kwazneski II, Douglas
collection PubMed
description Angiomyolipoma and PEComa are rare tumors descending from perivascular epithelial cells (PECs), with distinctive IHC, morphological, and ultrastructural features. The kidney is the most frequent site of origin, but not the only one; however, adrenal gland angiomyolipomas are extremely rare. We describe two cases being found in the adrenal glands. Given the paucity of literature on the subject, more information on this disease is necessary for diagnosis and treatment. Here, we describe two complete case reports, from presentation to treatment and follow-up, along with imaging and microscopic pathology samples, and provide a comprehensive review as to the history and current literature available regarding these extremely rare tumors.
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spelling pubmed-47798212016-03-20 Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors Kwazneski II, Douglas Merrill, Megan Young, Jessica Sell, Harry Case Rep Oncol Med Case Report Angiomyolipoma and PEComa are rare tumors descending from perivascular epithelial cells (PECs), with distinctive IHC, morphological, and ultrastructural features. The kidney is the most frequent site of origin, but not the only one; however, adrenal gland angiomyolipomas are extremely rare. We describe two cases being found in the adrenal glands. Given the paucity of literature on the subject, more information on this disease is necessary for diagnosis and treatment. Here, we describe two complete case reports, from presentation to treatment and follow-up, along with imaging and microscopic pathology samples, and provide a comprehensive review as to the history and current literature available regarding these extremely rare tumors. Hindawi Publishing Corporation 2016 2016-02-22 /pmc/articles/PMC4779821/ /pubmed/26998374 http://dx.doi.org/10.1155/2016/5204092 Text en Copyright © 2016 Douglas Kwazneski II et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kwazneski II, Douglas
Merrill, Megan
Young, Jessica
Sell, Harry
Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title_full Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title_fullStr Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title_full_unstemmed Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title_short Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors
title_sort angiomyolipoma and malignant pecoma: discussion of two rare adrenal tumors
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4779821/
https://www.ncbi.nlm.nih.gov/pubmed/26998374
http://dx.doi.org/10.1155/2016/5204092
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