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Doege-Potter syndrome associated to metastatic solitary fibrous tumor

Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cas...

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Autores principales: Andrade, Matheus de Oliveira, de Sousa, Nathália da Cruz, do Amaral, Paulo Siqueira, da Costa, Samantha Cabral Severino, de Lima, Luiz Guilherme Cernaglia Aureliano, Lourenço, Delmar Muniz, Feher, Olavo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital Universitário da Universidade de São Paulo 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9762516/
https://www.ncbi.nlm.nih.gov/pubmed/36569984
http://dx.doi.org/10.4322/acr.2021.412
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author Andrade, Matheus de Oliveira
de Sousa, Nathália da Cruz
do Amaral, Paulo Siqueira
da Costa, Samantha Cabral Severino
de Lima, Luiz Guilherme Cernaglia Aureliano
Lourenço, Delmar Muniz
Feher, Olavo
author_facet Andrade, Matheus de Oliveira
de Sousa, Nathália da Cruz
do Amaral, Paulo Siqueira
da Costa, Samantha Cabral Severino
de Lima, Luiz Guilherme Cernaglia Aureliano
Lourenço, Delmar Muniz
Feher, Olavo
author_sort Andrade, Matheus de Oliveira
collection PubMed
description Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.
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spelling pubmed-97625162022-12-23 Doege-Potter syndrome associated to metastatic solitary fibrous tumor Andrade, Matheus de Oliveira de Sousa, Nathália da Cruz do Amaral, Paulo Siqueira da Costa, Samantha Cabral Severino de Lima, Luiz Guilherme Cernaglia Aureliano Lourenço, Delmar Muniz Feher, Olavo Autops Case Rep Clinical Case Report Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge. Hospital Universitário da Universidade de São Paulo 2022-12-13 /pmc/articles/PMC9762516/ /pubmed/36569984 http://dx.doi.org/10.4322/acr.2021.412 Text en Copyright © 2022 The Authors. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Clinical Case Report
Andrade, Matheus de Oliveira
de Sousa, Nathália da Cruz
do Amaral, Paulo Siqueira
da Costa, Samantha Cabral Severino
de Lima, Luiz Guilherme Cernaglia Aureliano
Lourenço, Delmar Muniz
Feher, Olavo
Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title_full Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title_fullStr Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title_full_unstemmed Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title_short Doege-Potter syndrome associated to metastatic solitary fibrous tumor
title_sort doege-potter syndrome associated to metastatic solitary fibrous tumor
topic Clinical Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9762516/
https://www.ncbi.nlm.nih.gov/pubmed/36569984
http://dx.doi.org/10.4322/acr.2021.412
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