Table 2. Summary of the literature review.
| Author | Age at the time of diagnosis | Tumor | Genetic analysis | Surgery before sunitinib | Treatment | Wk of therapy | Outcome |
|---|---|---|---|---|---|---|---|
| Park KS and cols., 2009 | M (17 yr) | PHEO | NA | Yes | 37,5 mg/day for 7 weeks and 25 mg/day for 4 weeks | 11 | PR* after 7 weeks (according to 18FDG uptake) followed by SD* after 11 weeks |
| Jimenez C and cols., 2009 | F (32 yr) | PHEO (10.5 cm) | VHL | Yes | 50 mg/day 4 weeks on, 2 weeks off | 36 | PR* |
| Joshua AM and cols., 2009 | M (55 yr) | Abdominal PGL (14.4 cm) | SDHB | No (after six cycles) | 50/mg day 4 weeks on, 2 weeks off (before surgery); 37.5 mg/ day 4 weeks on, 2 weeks off (after surgery) | 48 | PR after 36 weeks followed by PD after 48 weeks (+ surgery) |
| M (28 yr) | Abdominal PGL (7 cm) | SDHB | Yes | 50/mg day 4 weeks on, 2 weeks off** | 40 | PR | |
| F (41 yr) | PHEO (15 cm) | Negative for SDHB, SDHD, RET and VHL | Yes | 50 mg/day 4 weeks on, 2 weeks off** | 40 | PR* | |
| Hahn NM and cols., 2009 | F (33 yr) | Abdominal PGL (17 cm) | SDHB | Yes | 50 mg/day 4 weeks on, 2 weeks off; 50 mg/day 2 weeks on, 1 week off | 16 | PD* |
| Cirillo F, 2010 | M (37 yr) | Abdominal PGL (17x14x9 cm) | NA | Yes | 50 mg/day 4 weeks on, 2 weeks off; 25 mg/day 4 weeks on, 2 weeks off; 25 mg/day 2 weeks on, 1 week off | 24 | SD* after 15 weeks followed by PD* after 24 weeks (+ octreotide LAR) |
| Zukauskaite R and cols., 2011 | M (31 yr) | PGL thoracic-lumbar region (10x15 cm) | No somatic mutations | Yes | 50 mg/day 4 weeks on, 2 weeks off | 24 | SD* after 12 weeks followed by PD* after 24 weeks |
| F (54 yr) | PHEO | Sporadic | Yes | 50 mg/day 4 weeks on, 2 weeks off reduced up to 12.5 mg/day | 68 | SD* after 40 weeks followed by PD* after 68 weeks | |
| Ayala-Ramirez M and cols., 2012 F (8); M (9) | (33 yr) | PHEO | VHL | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 24 | SD |
| (60 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 44 | PR | |
| (55 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 108 | SD | |
| (20 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | NA | SD | |
| (62 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 1.6 | PD | |
| (14 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 13 | PD | |
| (47 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 16 | PD | |
| (40 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 4 | PD | |
| (57 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | NA | NA (sunitinib was stopped due to toxicity) | |
| (60 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | NA | NA (sunitinib was stopped due to toxicity) | |
| (69 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | NA | NA (sunitinib was stopped due to toxicity) | |
| (27 yr) | PHEO | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | NA | SD | |
| (56 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 48 | PR | |
| (45 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 18 | PR | |
| (40 yr) | PGL | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 32 | SD | |
| (43 yr) | PHEO | SDHB | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 16.4 | PD | |
| (63 yr) | PHEO | Sporadic | No | 50 mg/day 4 weeks on, 2 weeks off or 37.5 mg/day continously or 37.5 mg/day 3 weeks on, 1 week off | 8.4 | PD | |
| Nemoto K and cols., 2012 | F (41 yr) | PHEO (10 cm) | NA | Yes | 50 mg/day 4 weeks on, 2 weeks off; 25 mg/day 2 weeks on, 2 weeks off | 26 | PR |
| Sun FK and cols., 2012 | M (32 yr) | PHEO (18 cm) | Negative for SDHB, SDHD, RET and VHL | Yes | 50 mg/day 4 weeks on, 2 weeks off; 37.5 mg/day 4 weeks on, 2 weeks off | 22 | Enlargement in the necrosis area of tumor with SD* |
| M (51 yr) | PHEO (12.9 cm) | Negative for SDHB, SDHD, RET and VHL | Yes | 50 mg/day 4 weeks on, 2 weeks off | 28 | Necrosis of the lesions at the CT scan (PR*) | |
| F (49 yr) | PHEO (5 cm) | Negative for SDHB, SDHD, RET and VHL | Yes | 50 mg/day 4 weeks on, 2 weeks off | 30 | PR* | |
| Prochilo T and cols., 2012 | F (35 yr) | Abdominal PGL | SDHB | Yes | 50 mg/day 4 weeks on, 2 weeks off; 37.5 mg daily 2 weeks on, 2 weeks off; 25 mg daily 2 weeks on, 1 week off | More than 36 | PR* after 12 weeks followed by SD* after 36 weeks and finally PD* (evaluated by 18FDG-PET) |
| Hata J and cols., 2014 | M (23 yr) | PHEO (8.7 cm) | NA | Yes | 50 mg/day 4 weeks on, 2 weeks off | 20 | SD (the authors not reported how many weeks after) followed by PD* after 20 weeks |
| M (60 yr) | PHEO (7.2 cm) | NA | Yes | 50 mg/day 4 weeks on, 2 weeks off** | 16 | SD* (the authors not reported how many weeks after) followed by PD* after 16 weeks | |
| Lebowitz-Amit R and cols., 2014 | M (51 yr) | Abdominal PGL (6.9x5.9 x 7.1 cm) | Negative for SDHB, SDHC, SDHD, TMEM127 and NF1 | Yes | 50 mg/day; 37.5 mg/day; 25/37.5 mg/day alternating | 24 | SD* |
| Bourcier ME and cols., 2013 | F (70 yr) | Abdominal PGL | NA | Yes | 50 mg/day 4 weeks on, 2 weeks off | 12 | CR |
| Our case | M (35yr) | Abdominal PGL | SDHB | yes | 25 mg/day 2 weeks on, 1 week off for the of time | 101 | PR after 12 weeks followed by SD after 32 weeks up to 54 weeks and PD after 78 weeks |
F: female; M: male; NA: not available; PGL: paraganglioma; PHEO: pheochromocytoma; * data deducted by the case description and not by RECIST evaluation; ** deducted data. Weeks are always reported from the beginning of sunitinib therapy.