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Br J Cancer. 2019 Jun;120(12):1113-1119. doi: 10.1038/s41416-019-0474-x. Epub 2019 May 20.

A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: the SNIPP trial.

Author information

1
Department of Medical Oncology and Haematology Princess Margaret Cancer Centre, University Health Network, 610 University Avenue, Toronto, Ontario M5G 2M9, Canada.
2
Endocrine Oncology Site Group, Princess Margaret Cancer Centre, University Health Network, 610 University Avenue, Toronto, Ontario M5G 2M9, Canada.
3
Department of Medical Oncology, Kinghorn Cancer Centre, 370 Victoria St, Darlinghurst, NSW, 2010, Australia.
4
Endocrinology Division, Department of Medicine, Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, Canada.
5
Department of Medical Oncology, Sheba Medical Center, Derech Sheba 2, Ramat-Gan, Israel.
6
Department of Medical Oncology, Tom Baker Cancer Centre, 1331 29 St NW, Calgary, Alberta T2N 4N2, Canada.
7
Department of Medical Oncology and Haematology Princess Margaret Cancer Centre, University Health Network, 610 University Avenue, Toronto, Ontario M5G 2M9, Canada. jennifer.knox@uhn.ca.

Abstract

BACKGROUND:

Pheochromocytoma (PCC) and paraganglioma (PGL) are uncommon neoplasms with high morbidity in advanced stages. Effective systemic treatments are limited.

METHODS:

A multisite phase 2 trial evaluated sunitinib in patients with progressive PCC/PGL. Patients received 50 mg orally for 4-6 weeks.

RESULTS:

Between May 2009 and May 2016, 25 patients were enroled. The median age was 50 years and 56% were male. Three patients (12%) received prior chemotherapy and 16 (64%) prior surgery. The DCR was 83% (95% CI: 61-95%) and median PFS 13.4 (95% CI: 5.3-24.6) months. Of 23 evaluable patients, 3 (13%) with germline mutations (SDHA, SDHB, RET) achieved a PR. The patient with mutated RET and MEN2A remains on treatment after 64 cycles. The median time on treatment was 12.4 (1-88.0) months. Grade 3 or 4 toxicities were as expected and manageable; fatigue (16%) and thrombocytopenia (16%) were most common. One patient with grade 3 hypertension and 2 with grade 3 cardiac events discontinued treatment.

CONCLUSION:

Although the primary endpoint of disease control was met, the overall response rate of sunitinib was low in unselected patients with progressive PCC/PGL. Patients with germline variants in RET or in the subunits of SDH may derive greatest benefit.

PMID:
31105270
DOI:
10.1038/s41416-019-0474-x

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