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An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report
Primary renal synovial sarcoma (PRSS) is an extremely rare malignancy. The diagnosis of PRSS is unforeseen due to the absence of clinical and radiological typical aspects. Here, we present a case of a 69-year-old male with complaints of hematuria and left lumbar pain. Abdominal-pelvic computed tomog...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9867795/ https://www.ncbi.nlm.nih.gov/pubmed/36694853 http://dx.doi.org/10.7759/cureus.33954 |
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author | Guimarães, Thiago Cristovão, Miguel Gião, Nuno Pinheiro, Hugo Campos Pinheiro, Luís |
author_facet | Guimarães, Thiago Cristovão, Miguel Gião, Nuno Pinheiro, Hugo Campos Pinheiro, Luís |
author_sort | Guimarães, Thiago |
collection | PubMed |
description | Primary renal synovial sarcoma (PRSS) is an extremely rare malignancy. The diagnosis of PRSS is unforeseen due to the absence of clinical and radiological typical aspects. Here, we present a case of a 69-year-old male with complaints of hematuria and left lumbar pain. Abdominal-pelvic computed tomography scan with contrast injection showed a solid mass of 8cm diameter in the left kidney and renal vein tumor thrombus. The patient was further subjected to robotic-assisted left radical nephrectomy and renal vein thrombectomy. We concomitantly performed left adrenalectomy and paraaortic lymphadenectomy. Immunohistochemical and genetic analysis revealed PRSS. This entity is characterized by abnormal chromosomal translocation t(X;18)(p11.2; q11.2) and consequently the characteristic SYT-SSX fusion gene. Due to the disease’s rarity and severity, diagnosis and management of PRSS rely upon a demanding and multidisciplinary approach. |
format | Online Article Text |
id | pubmed-9867795 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-98677952023-01-23 An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report Guimarães, Thiago Cristovão, Miguel Gião, Nuno Pinheiro, Hugo Campos Pinheiro, Luís Cureus Pathology Primary renal synovial sarcoma (PRSS) is an extremely rare malignancy. The diagnosis of PRSS is unforeseen due to the absence of clinical and radiological typical aspects. Here, we present a case of a 69-year-old male with complaints of hematuria and left lumbar pain. Abdominal-pelvic computed tomography scan with contrast injection showed a solid mass of 8cm diameter in the left kidney and renal vein tumor thrombus. The patient was further subjected to robotic-assisted left radical nephrectomy and renal vein thrombectomy. We concomitantly performed left adrenalectomy and paraaortic lymphadenectomy. Immunohistochemical and genetic analysis revealed PRSS. This entity is characterized by abnormal chromosomal translocation t(X;18)(p11.2; q11.2) and consequently the characteristic SYT-SSX fusion gene. Due to the disease’s rarity and severity, diagnosis and management of PRSS rely upon a demanding and multidisciplinary approach. Cureus 2023-01-19 /pmc/articles/PMC9867795/ /pubmed/36694853 http://dx.doi.org/10.7759/cureus.33954 Text en Copyright © 2023, Guimarães et al. https://creativecommons.org/licenses/by/3.0/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 author and source are credited. |
spellingShingle | Pathology Guimarães, Thiago Cristovão, Miguel Gião, Nuno Pinheiro, Hugo Campos Pinheiro, Luís An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title | An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title_full | An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title_fullStr | An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title_full_unstemmed | An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title_short | An Extremely Rare and Demanding Diagnosis of Primary Renal Synovial Sarcoma: A Case Report |
title_sort | extremely rare and demanding diagnosis of primary renal synovial sarcoma: a case report |
topic | Pathology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9867795/ https://www.ncbi.nlm.nih.gov/pubmed/36694853 http://dx.doi.org/10.7759/cureus.33954 |
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