Continued Tumor Reduction of Metastatic Pheochromocytoma/Paraganglioma Harboring Succinate Dehydrogenase Subunit B Mutations with Cyclical Chemotherapy

被引:0
作者
Irfan Jawed
Margarita Velarde
Roland Därr
Katherine I. Wolf
Karen Adams
Aradhana M. Venkatesan
Sanjeeve Balasubramaniam
Marianne S. Poruchynsky
James C. Reynolds
Karel Pacak
Tito Fojo
机构
[1] National Institutes of Health,Medical Oncology, National Cancer Institute
[2] National Institutes of Health,Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development
[3] MD Anderson Cancer Center,Section of Abdominal Imaging, Department of Diagnostic Radiology
[4] National Institutes of Health,Nuclear Medicine Division, Department of Radiology and Imaging Sciences, Clinical Center
来源
Cellular and Molecular Neurobiology | 2018年 / 38卷
关键词
Pheochromocytoma/paraganglioma; Succinate dehydrogenase; Cyclophosphamide; Vincristine; Dacarbazine;
D O I
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学科分类号
摘要
Patients harboring germline mutations in the succinate dehydrogenase complex subunit B (SDHB) gene present with pheochromocytomas and paragangliomas (PPGL) that are more likely malignant and clinically aggressive. The combination chemotherapy cyclophosphamide, vincristine, and dacarbazine (CVD) was retrospectively evaluated in patients with SDHB-associated metastatic PPGL.Query Twelve metastatic PPGL patients harboring SDHB mutations/polymorphisms with undetectable SDHB immunostaining were treated with CVD. CVD therapy consisted of 750 mg/m2 cyclophosphamide with 1.4 mg/m2 vincristine on day 1 and 600 mg/m2 dacarbazine on days 1 and 2, every 21–28 days. Treatment outcome was determined by RECIST criteria as well as determination of response duration and progression-free and overall survivals. A median of 20.5 cycles (range 4–41) was administered. All patients had tumor reduction (12–100% by RECIST). Complete response was seen in two patients, while partial response was observed in 8. The median number of cycles to response was 5.5. Median duration of response was 478 days, with progression-free and overall survivals of 930 and 1190 days, respectively. Serial [18F]-fluorodeoxyglucose positron emission tomography and computed tomography imaging demonstrated continued incremental reduction in maximal standardized uptake values (SUVmax) values in 26/30 lesions. During treatment administration, the median SUV decreased from > 25 to < 6, indicating the efficacy of chemotherapy over a prolonged period of time. Prolonged therapy results in continued incremental tumor reduction, and is consistent with persistent drug sensitivity. CVD chemotherapy is recommended to be considered part of the initial management in patients with metastatic SDHB-related PPGL.
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页码:1099 / 1106
页数:7
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