Table 1.

Clinical characteristics of patients treated with metronomic TMZ and high-dose lanreotide.

Patient no.	Sex	Age at diagnosis Primary	Ki 67 index, %	Age at mPGL diagnosis	Site of metastases	SDHB mutation	MGMT methylation	Treatment cycles, n	Biochemical response	Therapies	PFS to prior therapies, mo	Best response to prior therapies	PERCIST 1.0	PFS, mo	Survival status
1	M	58	20	60	Bone, soft tissue	Yes	Yes	27	PRa	Surgery	24		PMRb	NR	Alive
										Thermoablation
										Sunitinib	2	PMD
										2 monthly lanreotide + CVD	6	PMR
2	F	46	5	46	Bone, soft tissue	Yes	Yes	13	CRa	CVD	8	PMR	PMRb	13	Alive
										High-dose lanreotide	15	PMR
										Surgery
										Thermoablation

Abbreviations: CR, complete response; CVD, cyclophosphamide, vincristine, and dacarbazine; F, female; M, male; MGMT, O6-methylguanine-DNA methyltransferase; mPGL, malignant pheochromocytoma and paraganglioma; PERCIST, positron emission tomography response criteria in solid tumors; PFS, progression-free survival; PMD, progressive metabolic disease; PMR, partial metabolic response; PR, partial response; SDHB, succinate dehydrogenase subunit B; TMZ, temozolomide; NR, not reached.

^aBiochemical response in patient 1 was based on urinary normetanephrine levels obtained at baseline and after 4 and 11 cycles of chemotherapy. Urinary normetanephrine levels were used to monitor disease progression because the biochemical phenotype of the tumor was noradrenergic (mainly producing norepinephrine and normetanephrine). In patient 2, biochemical response was based on plasma chromogranin A levels. Other biochemical markers were not available at regular time intervals.

^bBoth patients demonstrated PMR as their best response to this treatment regimen on PERCIST 1.0 criteria. While patient 1 continued to demonstrate a stabilized PMR after 21 cycles, patient 2 demonstrated progressive metabolic disease (PMD) after 13 cycles with dissociated response.