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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Autores principales: David Strauss, Andres Correa, Yulan Gong, Andreas Karachristos, Alexander Kutikov
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Lenguaje:EN
Publicado: Elsevier 2022
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Acceso en línea:https://doaj.org/article/df5fdb05953c404eb70c4b417988e9fb
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spelling 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)
institution DOAJ
collection DOAJ
language EN
topic Retroperitoneal
Giant paraganglioma
Succinate dehydrogenase mutation
Diseases of the genitourinary system. Urology
RC870-923
spellingShingle 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
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