Table 2.
Main findings from studies performing peptide receptor radionuclide therapy in patients with inoperable/metastatic pheochromocytoma or paraganglioma (case reports were excluded).
| Authors | Year | Country | Type of study | No. of PPGL patients | Progressive disease at baseline | Radiopharmaceutical | No. of cycles | Concomitant treatment | Data used for response assessment | No. of responders (%) | No. of responders or stable disease (%) | Median PFS (months) | Median OS (months) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Kolasinska-Cwikla | 2019 | Poland | Prospective single centre | 13 | 13 (100%) | 90Y-DOTATATE | *2.5 | - | Morphological | 1/12 (8%) |
10/12 (83%) |
35.0 | 68.0 |
| Vyakaranam et al. | 2019 | Sweden | Retrospective single centre | 22 | 9 (41%) | 177Lu-DOTATATE | 3–11 | - | Morphological, biochemical and clinical data | 2/22 (9%) |
22/22 (100%) |
21.6 | 49.6 |
| Zandee et al. | 2019 | Netherlands | Retrospective single centre | 30 | 20 (67%) | 177Lu-DOTATATE | 4 | - | Morphological and clinical data | 7/30 (23%) |
27/30 (90%) |
30.0 | N.A. |
| Yadav et al. | 2019 | India | Retrospective single centre | 25 | 21 (84%) | 177Lu-DOTATATE | 2–8 | Ch (100%) | SSA PET/CT, morphological, biochemical and clinical data | 7/25 (28%) |
21/25 (84%) |
32.0 | N.A. |
| Kong et al. | 2017 | Australia Israel |
Retrospective bicentric | 20 | 6 (30%) | 177Lu-DOTATATE | 1–4 | Ch (45%) | SSA PET/CT, morphological, biochemical and clinical data | 8/17 (47%) |
15/17 (88%) |
39.0 | N.A. |
| Nastos et al. | 2017 | UK | Retrospective single centre | 13 | 13 (100%) | 177Lu-/90Y-DOTATATE | 1–4 | Ch, RT or SSA in some cases | Morphological, biochemical and clinical data | NA | 13/13 (100%) |
38.5 | 60.8 |
| Pinato et al. | 2016 | UK | Case series | 5 | 5 (100%) | 177Lu-DOTATATE | 1–4 | - | SSA PET/CT and morphological data | 1/5 (20%) |
4/5 (80%) |
17.0 | N.A. |
| Estevao et al. | 2015 | Portugal | Retrospective single centre | 14 | 4 (29%) | 177Lu-DOTATATE | 3 | - | SSA PET/CT and clinical data | 10/14 (71%) |
10/14 (71%) |
N.A. | N.A. |
| Puranik et al. | 2015 | Germany | Prospective single centre | 9 | N.A. | 177Lu-/90Y-DOTATATE/ DOTATOC | 2–4 | - | SSA PET/CT, morphological and clinical data | 4/9 (44%) |
9/9 (100%) |
N.A. | N.A. |
| Zovato et al. | 2012 | Italy | Case series | 4 | 4 (100%) | 177Lu-DOTATATE | 3–5 | - | SSA scintigraphy, morphological and clinical data | 2/4 (50%) |
4/4 (100%) |
N.A. | N.A. |
| Imhof et al.** | 2011 | Switzerland | Prospective single centre | 39 | 39 (100%) | 90Y-DOTATOC | 1–10 | - | SSA scintigraphy, morphological, biochemical and clinical data | 7/39 (18%) |
NA | N.A. | N.A. |
| Forrer et al. | 2008 | Switzerland | Retrospective single centre | 28 | 28 (100%) | 177Lu-/90Y-DOTATOC | 1–4 | - | Morphological, biochemical and clinical data | 7/28 (25%) |
20/28 (71%) |
N.A. | N.A. |
| van Essen et al.*** | 2006 | Netherlands | Retrospective single centre | 12 | 4 (33%) | 177Lu-DOTATATE | 4 | - | Morphological, biochemical and clinical data | 2/11 (18%) |
8/11 (73%) |
N.A. | N.A. |
study not included in the meta-analysis for possible patient overlap with the study of Forrer et al.;
study not included in the meta-analysis for possible patient overlap with the study of Zandee et al.; OS: overall survival; N.A.: not available; PFS: progression-free survival; PPGL: pheochromocytoma/paraganglioma; PRRT: peptide receptor radionuclide therapy; Ch: chemotherapy; PET/CT: positron emission tomography-computed tomography; RT: radiation therapy; SSA: somatostatin analog. Note: response assessment criteria usually included various combinations of imaging (morphological and/or 68Ga-DOTA-SSA PET/CT), biochemical, and clinical data as mentioned in the table.