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Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective

Solitary fibrous tumors are mesenchymal lesions that arise at a variety of sites, most commonly the pleura. Most patients are asymptomatic at diagnosis, with lesions being detected incidentally. Nevertheless, some patients present due to symptoms from local tumor compression (eg. of the airways and p...

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Autores principales: Saynak, Mert, Veeramachaneni, Nirmal K., Hubbs, Jessica L., Okumuş, Dilruba, Marks, Lawrence B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Galenos Publishing 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5450857/
https://www.ncbi.nlm.nih.gov/pubmed/28443588
http://dx.doi.org/10.4274/balkanmedj.2017.0350
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author Saynak, Mert
Veeramachaneni, Nirmal K.
Hubbs, Jessica L.
Okumuş, Dilruba
Marks, Lawrence B.
author_facet Saynak, Mert
Veeramachaneni, Nirmal K.
Hubbs, Jessica L.
Okumuş, Dilruba
Marks, Lawrence B.
author_sort Saynak, Mert
collection PubMed
description Solitary fibrous tumors are mesenchymal lesions that arise at a variety of sites, most commonly the pleura. Most patients are asymptomatic at diagnosis, with lesions being detected incidentally. Nevertheless, some patients present due to symptoms from local tumor compression (eg. of the airways and pulmonary parenchyma). Furthermore, radiological methods are not always conclusive in making a diagnosis, and thus, pathological analysis is often required. In the past three decades, immunohistochemical techniques have provided a gold standard in solitary fibrous tumor diagnosis. The signature marker of solitary fibrous tumor is the presence of the NAB2-STAT6 fusion that can be reliably detected with a STAT6 antibody. While solitary fibrous tumors are most often benign, they can be malignant in 10-20% of the cases. Unfortunately, histological parameters are not always predictive of benign vs malignant solitary fibrous tumors. As solitary fibrous tumors are generally regarded as relatively chemoresistant tumors; treatment is often limited to localized treatment modalities. The optimal treatment of solitary fibrous tumors appears to be complete surgical resection for both primary and local recurrent disease. However, in cases of suboptimal resection, large disease burden, or advanced recurrence, a multidisciplinary approach may be preferable. Specifically, radiotherapy for inoperable local disease can provide palliation/shrinkage. Given their sometimes -unpredictable and often- protracted clinical course, long-term follow-up post-resection is recommended.
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spelling pubmed-54508572017-06-01 Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective Saynak, Mert Veeramachaneni, Nirmal K. Hubbs, Jessica L. Okumuş, Dilruba Marks, Lawrence B. Balkan Med J Invited Review Solitary fibrous tumors are mesenchymal lesions that arise at a variety of sites, most commonly the pleura. Most patients are asymptomatic at diagnosis, with lesions being detected incidentally. Nevertheless, some patients present due to symptoms from local tumor compression (eg. of the airways and pulmonary parenchyma). Furthermore, radiological methods are not always conclusive in making a diagnosis, and thus, pathological analysis is often required. In the past three decades, immunohistochemical techniques have provided a gold standard in solitary fibrous tumor diagnosis. The signature marker of solitary fibrous tumor is the presence of the NAB2-STAT6 fusion that can be reliably detected with a STAT6 antibody. While solitary fibrous tumors are most often benign, they can be malignant in 10-20% of the cases. Unfortunately, histological parameters are not always predictive of benign vs malignant solitary fibrous tumors. As solitary fibrous tumors are generally regarded as relatively chemoresistant tumors; treatment is often limited to localized treatment modalities. The optimal treatment of solitary fibrous tumors appears to be complete surgical resection for both primary and local recurrent disease. However, in cases of suboptimal resection, large disease burden, or advanced recurrence, a multidisciplinary approach may be preferable. Specifically, radiotherapy for inoperable local disease can provide palliation/shrinkage. Given their sometimes -unpredictable and often- protracted clinical course, long-term follow-up post-resection is recommended. Galenos Publishing 2017-05 2017-05-15 /pmc/articles/PMC5450857/ /pubmed/28443588 http://dx.doi.org/10.4274/balkanmedj.2017.0350 Text en © Copyright 2017, Trakya University Faculty of Medicine http://creativecommons.org/licenses/by/2.5/ Balkan Medical Journal
spellingShingle Invited Review
Saynak, Mert
Veeramachaneni, Nirmal K.
Hubbs, Jessica L.
Okumuş, Dilruba
Marks, Lawrence B.
Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title_full Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title_fullStr Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title_full_unstemmed Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title_short Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective
title_sort solitary fibrous tumors of chest: another look with the oncologic perspective
topic Invited Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5450857/
https://www.ncbi.nlm.nih.gov/pubmed/28443588
http://dx.doi.org/10.4274/balkanmedj.2017.0350
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