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
Formato: article
Lenguaje:EN
Publicado: Elsevier 2022
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Acceso en línea:https://doaj.org/article/df5fdb05953c404eb70c4b417988e9fb
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Sumario: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.