{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T15:52:14Z","timestamp":1753890734730,"version":"3.41.2"},"reference-count":36,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,12,18]],"date-time":"2024-12-18T00:00:00Z","timestamp":1734480000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Succinate dehydrogenase subunit B (SDHB)-mutated paragangliomas (PGLs) are rare neuroendocrine tumors characterized by increased malignancy, readily metastasizing, and poorer prognosis. Here we report a case of SDHB-mutated metastatic PGL, wherein the patient showed significant tumor shrinkage and complete symptom remission following chemotherapy. We aim to contribute additional evidence to the existing knowledge associated with SDHB-mutated PGLs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case report<\/jats:title><jats:p>A 40-year-old male patient presented with recurrent hypoglycemia and hypertension crisis. Imaging revealed a huge left retroperitoneal tumor and multiple diffuse metastases in lungs. Catecholamine was also elevated, aligning with a diagnosis of metastatic PGL. Pathology also confirmed this diagnosis. Additionally, the immunohistochemistry indicated negative expression of SDHB and gene test showed somatic SDHB mutation. Given the SDHB mutation, cyclophosphamide-vincristine-dacarbazine (CVD) chemotherapy was initiated in critical conditions. Subsequently, a significant tumor shrinkage and complete biochemical response were observed after two treatment cycles. In September 2024, CT scan revealed new pulmonary lesions. The progression-free survival (PFS) with CVD chemotherapy was 24 months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This report reviews the distinct clinical and biochemical characteristics and treatment approaches of SDHB-mutated paragangliomas, emphasizing that the significance of incorporating both genetic testing and immunohistochemical analysis in clinical practice.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fendo.2024.1483516","type":"journal-article","created":{"date-parts":[[2024,12,18]],"date-time":"2024-12-18T05:11:16Z","timestamp":1734498676000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Durable and deep response to CVD chemotherapy in SDHB-mutated metastatic paraganglioma: case report"],"prefix":"10.3389","volume":"15","author":[{"given":"Chenyan","family":"Zhang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuanfeng","family":"Wei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ke","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Cao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,12,18]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.beem.2020.101416","article-title":"An overview of 20 years of genetic studies in pheochromocytoma and paraganglioma","volume":"34","author":"Buffet","year":"2020","journal-title":"Best Pract Res Clin Endocrinol 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