Since promoter methylation is widespread in SDH-deficient GISTs, alkylating agents may have a potential role in this tumor subgroup.132A phase II trial on temozolomide in advanced SDH-GISTs is still ongoing; a prolonged disease stability after 18 consecutive cycles of temozolomide has been recently reported in a female metastatic and progressive SDH-deficient GIST.123. The gene discussed is SDHB; the disease is neoplasm.