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Eccrine Porocarcinoma: A Review of the Literature

Eccrine porocarcinoma (EPC) constitutes a rare malignant adnexal tumor, which accounts for about 0.005–0.01% of all cutaneous malignancies. It may develop de novo or arise from an eccrine poroma, after a latency period of years or even decades. Accumulating data suggest that specific oncogenic drive...

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Autores principales: Tsiogka, Aikaterini, Koumaki, Dimitra, Kyriazopoulou, Maria, Liopyris, Konstantinos, Stratigos, Alexander, Gregoriou, Stamatios
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10137440/
https://www.ncbi.nlm.nih.gov/pubmed/37189532
http://dx.doi.org/10.3390/diagnostics13081431
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author Tsiogka, Aikaterini
Koumaki, Dimitra
Kyriazopoulou, Maria
Liopyris, Konstantinos
Stratigos, Alexander
Gregoriou, Stamatios
author_facet Tsiogka, Aikaterini
Koumaki, Dimitra
Kyriazopoulou, Maria
Liopyris, Konstantinos
Stratigos, Alexander
Gregoriou, Stamatios
author_sort Tsiogka, Aikaterini
collection PubMed
description Eccrine porocarcinoma (EPC) constitutes a rare malignant adnexal tumor, which accounts for about 0.005–0.01% of all cutaneous malignancies. It may develop de novo or arise from an eccrine poroma, after a latency period of years or even decades. Accumulating data suggest that specific oncogenic drivers and signaling pathways may be implicated in its tumorigenesis, while recent data have demonstrated a high overall mutation rate attributed to UV exposure. Diagnosis may be challenging and should rely on the combination of clinical, dermoscopical, histopathological and immunohistochemical findings. The literature is controversial regarding tumor behavior and prognosis and, therefore, there is no consensus on its surgical management, utility of lymph-node biopsy and further adjuvant or systemic treatment. However, recent advances in tumorigenesis of EPC may aid in the development of novel treatment strategies, which could improve survival of advanced or metastatic disease, such as immunotherapy. This review presents an update of the epidemiology, pathogenesis and clinical presentation of EPC and summarizes current data on diagnostic evaluation and management of this rare cutaneous malignancy.
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spelling pubmed-101374402023-04-28 Eccrine Porocarcinoma: A Review of the Literature Tsiogka, Aikaterini Koumaki, Dimitra Kyriazopoulou, Maria Liopyris, Konstantinos Stratigos, Alexander Gregoriou, Stamatios Diagnostics (Basel) Review Eccrine porocarcinoma (EPC) constitutes a rare malignant adnexal tumor, which accounts for about 0.005–0.01% of all cutaneous malignancies. It may develop de novo or arise from an eccrine poroma, after a latency period of years or even decades. Accumulating data suggest that specific oncogenic drivers and signaling pathways may be implicated in its tumorigenesis, while recent data have demonstrated a high overall mutation rate attributed to UV exposure. Diagnosis may be challenging and should rely on the combination of clinical, dermoscopical, histopathological and immunohistochemical findings. The literature is controversial regarding tumor behavior and prognosis and, therefore, there is no consensus on its surgical management, utility of lymph-node biopsy and further adjuvant or systemic treatment. However, recent advances in tumorigenesis of EPC may aid in the development of novel treatment strategies, which could improve survival of advanced or metastatic disease, such as immunotherapy. This review presents an update of the epidemiology, pathogenesis and clinical presentation of EPC and summarizes current data on diagnostic evaluation and management of this rare cutaneous malignancy. MDPI 2023-04-16 /pmc/articles/PMC10137440/ /pubmed/37189532 http://dx.doi.org/10.3390/diagnostics13081431 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Tsiogka, Aikaterini
Koumaki, Dimitra
Kyriazopoulou, Maria
Liopyris, Konstantinos
Stratigos, Alexander
Gregoriou, Stamatios
Eccrine Porocarcinoma: A Review of the Literature
title Eccrine Porocarcinoma: A Review of the Literature
title_full Eccrine Porocarcinoma: A Review of the Literature
title_fullStr Eccrine Porocarcinoma: A Review of the Literature
title_full_unstemmed Eccrine Porocarcinoma: A Review of the Literature
title_short Eccrine Porocarcinoma: A Review of the Literature
title_sort eccrine porocarcinoma: a review of the literature
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10137440/
https://www.ncbi.nlm.nih.gov/pubmed/37189532
http://dx.doi.org/10.3390/diagnostics13081431
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