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Favorable Outcome in Patients with Pheochromocytoma and Paraganglioma Treated with 177Lu-DOTATATE

1
Section of Radiology, Molecular Imaging, Department of Surgical Sciences, Uppsala University, Akademiska Sjukhuset, SE-751 85 Uppsala, Sweden
2
Department of Medical Sciences, Uppsala University, Uppsala University Hospital, SE-751 85 Uppsala, Sweden
3
Section of Endocrine Surgery, Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, SE-751 85 Uppsala, Sweden
4
Department of Nuclear Medicine, Sahlgrenska University Hospital, SE-413 45 Gothenburg, Sweden
5
Department of Gastroenterology, Oslo University Hospital, Rikshospitalet, 0372 Oslo, Norway
*
Author to whom correspondence should be addressed.
Cancers 2019, 11(7), 909; https://doi.org/10.3390/cancers11070909
Received: 10 June 2019 / Revised: 24 June 2019 / Accepted: 26 June 2019 / Published: 28 June 2019
(This article belongs to the Special Issue Pheochromocytoma (PHEO) and Paraganglioma (PGL))
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Abstract

Peptide receptor radiotherapy (PRRT) with 177Lu-DOTATATE has emerged as a promising therapy for neuroendocrine tumors (NETs). This retrospective cohort study aimed to assess the outcome of PRRT for 22 patients with histopathologically confirmed pheochromocytoma (PCC) and paraganglioma (PGL), of which two were localized and 20 metastatic. Radiological response utilized response evaluation criteria in solid tumors 1.1 and toxicity was graded according to common terminology criteria for adverse events version 4. Median 4 (range 3–11) 7.4 GBq cycles of 177Lu-DOTATATE were administered as first-line therapy (n = 13) or because of progressive disease (n = 9). Partial response (PR) was achieved in two and stable disease (SD) in 20 patients. The median overall survival (OS) was 49.6 (range 8.2–139) months and median progression-free survival (PFS) was 21.6 (range 6.7–138) months. Scintigraphic response >50% was achieved in 9/19 (47%) patients. Biochemical response (>50% decrease) of chromogranin A was found in 6/15 (40%) patients and of catecholamines in 3/12 (25%) patients. Subgroup analysis showed Ki-67 <15% associated with longer OS (p = 0.013) and PFS (p = 0.005). PRRT as first-line therapy was associated with increased OS (p = 0.041). No hematological or kidney toxicity grade 3–4 was registered. 177Lu-DOTATATE therapy was associated with favorable outcome and low toxicity. High Ki-67 (≥15%) and PRRT received because of progression on previous therapy could constitute negative predictive factors for OS. View Full-Text
Keywords: pheochromocytoma; paraganglioma; 177Lu-DOTATATE; peptide receptor radiotherapy; PRRT; neuroendocrine tumor; NET; PCC; PGL pheochromocytoma; paraganglioma; 177Lu-DOTATATE; peptide receptor radiotherapy; PRRT; neuroendocrine tumor; NET; PCC; PGL
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Vyakaranam, A.R.; Crona, J.; Norlén, O.; Granberg, D.; Garske-Román, U.; Sandström, M.; Fröss-Baron, K.; Thiis-Evensen, E.; Hellman, P.; Sundin, A. Favorable Outcome in Patients with Pheochromocytoma and Paraganglioma Treated with 177Lu-DOTATATE. Cancers 2019, 11, 909.

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