Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas
Paragangliomas are rare neuroendocrine tumors that can vary in size and metabolic activity. We report a case of giant bilateral malignant retroperitoneal paragangliomas (PGL) in a patient with germline succinate dehydrogenase B (SDHB) mutation. This patient, who presented in an emaciated and debilit...
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2022
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oai:doaj.org-article:df5fdb05953c404eb70c4b417988e9fb2021-12-04T04:34:18ZManagement of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas2214-442010.1016/j.eucr.2021.101950https://doaj.org/article/df5fdb05953c404eb70c4b417988e9fb2022-01-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2214442021003909https://doaj.org/toc/2214-4420Paragangliomas are rare neuroendocrine tumors that can vary in size and metabolic activity. We report a case of giant bilateral malignant retroperitoneal paragangliomas (PGL) in a patient with germline succinate dehydrogenase B (SDHB) mutation. This patient, who presented in an emaciated and debilitated state, was managed with adrenergic blockade followed by radical primary surgery. After being metabolically and radiographically disease free for 4 years, he underwent salvage resection for recurrent retroperitoneal disease and palliative radiation to a site of solidary vertebral metastasis. We review incidence and prognosis of metastatic PGL.David StraussAndres CorreaYulan GongAndreas KarachristosAlexander KutikovElsevierarticleRetroperitonealGiant paragangliomaSuccinate dehydrogenase mutationDiseases of the genitourinary system. UrologyRC870-923ENUrology Case Reports, Vol 40, Iss , Pp 101950- (2022) |
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Retroperitoneal Giant paraganglioma Succinate dehydrogenase mutation Diseases of the genitourinary system. Urology RC870-923 |
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Retroperitoneal Giant paraganglioma Succinate dehydrogenase mutation Diseases of the genitourinary system. Urology RC870-923 David Strauss Andres Correa Yulan Gong Andreas Karachristos Alexander Kutikov Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
description |
Paragangliomas are rare neuroendocrine tumors that can vary in size and metabolic activity. We report a case of giant bilateral malignant retroperitoneal paragangliomas (PGL) in a patient with germline succinate dehydrogenase B (SDHB) mutation. This patient, who presented in an emaciated and debilitated state, was managed with adrenergic blockade followed by radical primary surgery. After being metabolically and radiographically disease free for 4 years, he underwent salvage resection for recurrent retroperitoneal disease and palliative radiation to a site of solidary vertebral metastasis. We review incidence and prognosis of metastatic PGL. |
format |
article |
author |
David Strauss Andres Correa Yulan Gong Andreas Karachristos Alexander Kutikov |
author_facet |
David Strauss Andres Correa Yulan Gong Andreas Karachristos Alexander Kutikov |
author_sort |
David Strauss |
title |
Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
title_short |
Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
title_full |
Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
title_fullStr |
Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
title_full_unstemmed |
Management of SHDB positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
title_sort |
management of shdb positive patient with metastatic bilateral giant retroperitoneal paragangliomas |
publisher |
Elsevier |
publishDate |
2022 |
url |
https://doaj.org/article/df5fdb05953c404eb70c4b417988e9fb |
work_keys_str_mv |
AT davidstrauss managementofshdbpositivepatientwithmetastaticbilateralgiantretroperitonealparagangliomas AT andrescorrea managementofshdbpositivepatientwithmetastaticbilateralgiantretroperitonealparagangliomas AT yulangong managementofshdbpositivepatientwithmetastaticbilateralgiantretroperitonealparagangliomas AT andreaskarachristos managementofshdbpositivepatientwithmetastaticbilateralgiantretroperitonealparagangliomas AT alexanderkutikov managementofshdbpositivepatientwithmetastaticbilateralgiantretroperitonealparagangliomas |
_version_ |
1718372999863730176 |