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CASE REPORT article

Front. Endocrinol.
Sec. Neuroendocrine Science
Volume 15 - 2024 | doi: 10.3389/fendo.2024.1483516
This article is part of the Research Topic Recent Advances in Pheochromocytoma and Paraganglioma: Molecular Pathogenesis, Clinical Impacts, and Therapeutic Perspective - Volume II View all 8 articles

Durable and deep response to CVD chemotherapy in SDHB-mutated metastatic paraganglioma: case report

Provisionally accepted
Chenyan Zhang Chenyan Zhang Yuanfeng Wei Yuanfeng Wei Ke Cheng Ke Cheng *Dan Cao Dan Cao *
  • Department of Abdominal Oncology, Cancer Center, West China Hospital, Sichuan University, Chengdu, China

The final, formatted version of the article will be published soon.

    Introduction 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. Case report 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 progressionfree survival (PFS) with CVD chemotherapy was 24 months. Discussion 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.

    Keywords: SDHB-mutation, metastatic paraganglioma, CVD chemotherapy, case report, Hypoglycemia

    Received: 28 Oct 2024; Accepted: 04 Dec 2024.

    Copyright: © 2024 Zhang, Wei, Cheng and Cao. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Ke Cheng, Department of Abdominal Oncology, Cancer Center, West China Hospital, Sichuan University, Chengdu, China
    Dan Cao, Department of Abdominal Oncology, Cancer Center, West China Hospital, Sichuan University, Chengdu, China

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