CHEMOTHERAPY FOR PARAGANGLIOMAS

Eur J Endocrinol. 2012 Mar 19. [Epub ahead of print]

A SDHB-malignant paraganglioma with dramatic response to temozolomide-capecitabine.

Nozieres C, Walter T, Joly MO, Giraud S, Scoazec JY, Borson-Chazot F, Simon C, Riou JP, Lombard-Bohas C.

Source

C Nozieres, Oncologie médicale, Hôpital Edouard Herriot, Lyon, France.

Abstract

Ten percent of paragangliomas are malignant and one third occurs in a genetic background. We report a case of SDHB-related malignant paraganglioma with dramatic response to temozolomide and capecitabine regimen (decrease in tumor size of 70% with RECIST criteria). Tumor cells harboured a new mutation in succinate dehydrogenase subunit B (SDHB) gene and showed aberrant hypermethylation of MGMT promoter. Our report suggests the importance of molecular predictive factors of response for the selection of chemotherapeutic as well as targeted agents. This observation points out to possible genotype-response to treatment relationships which could help to design tailor-made treatments in the future.