Table 1.
Clinicopathologic summary of the 66 SDH-deficient GISTs
| Case | Age | Sex | Size (cm) | Mitoses/50 HPFs | Prognostic group | Recurrences, metastases and additional findings | Status at last follow-up | Length of follow-up |
|---|---|---|---|---|---|---|---|---|
| 1 | 8 | F | 8 largest, multiple | 18 | 6a | Abdominal metastases, 18 yrs | AWD | 18 years |
| 2 | 9 | F | 2.5 cm | 1 | 3a | Gastric recurrence, 5.0 years. Regional abdominal metastasis at 10 years, exicised following 6 month of imatinib | ANED | 16 years |
| 3 | 10 | F | 5 + 3 cm, two | 10 | 5 | ANED | 24.1 years | |
| 4 | 10 | F | 5.7 and a smaller nodule | 3 | 3a | ANED | 8.6 years | |
| 5 | 10 | F | 6 + 3 cm, two | 11 | 6a | Lymph node metastasis at presentation | ANED | 1. 8 years |
| 6 | 10 | F | >10 | 8 | 6b | Gastric recurrence, 13 years, gastrectomy | ANED | 29.4 years |
| 7 | 11 | F | 3 | 16 | 5 | Gastric recurrence at 3 years | ANED | 20.8 years |
| 8 | 11 | F | 5 | 0 | 2 | ANED | 7 years | |
| 9 | 12 | F | 2.7 | 6 | 5 | ANED | 9.7 years | |
| 12 | F | 4 | 3 | 2 | ANED | 6.0 years | ||
| 11 | 12 | F | 5 | 18 | 5 | Pulmonary chondroma one year later (Carney triad); Gastric recurrences, 9 and 33 years, total gastrectomy | ANED | 41.2 years |
| 12 | 12 | F | 8.5 | 57 | 6a | Liver metastases at presentation | LTF | |
| 13 | 13 | F | 4.3 | 4 | 2 | Liver and mesocolic metastases, time of diagnosis unknown | DOD | 6.5 years |
| 14 | 14 | F | 3.5 | 2 | 2 | ANED | 28 years | |
| s15 | 14 | F | 4.5 | 7 | 5 | ANED | 13 years | |
| 16 | 14 | F | 9 | 6 | 6a | Pulmonary chondroma at presentation (Carney triad). Gastric recurrence, 4 years | ANED | 9 years |
| 17 | 14 | F | Multiple, 0.5–4.5 cm | 8 | 5 | Liver metastasis, 2.4 years. Received imatinib mesylate for 5 years | AWD | 8.8 years |
| 18 | 14 | M | 7 | 9 | 6a | ANED | 4.9 years | |
| 19 | 15 | F | 3.5, another in fundus | 6 | 5 | Peritoneal micrometastases at presentation Subtotal gastrectomy for residual disease6 months later | ANED | 17.4 years |
| 20 | 16 | F | Unknown, recurrence 4.5 cm | 3 (2nd rec) | * | Gastric recurrences 17, 23, and 24 years later | AWD | 24 years |
| 21 | 16 | M | 8 | 2 | 3a | Liver metastasis, 22 years later | DOD | 23.3 years |
| 22 | 16 | M | Unknown | ND | * | Liver metastasis, 14 years later. Received imatinib for 19 years after primary, for 7 years | DOD | 27 years |
| 23 | 17 | M | 2.7 | 6 | 5 | Liver metastasis at presentation. Gastric recurrence 3 years later | AWD | 18.8 years |
| 24 | 18 | F | 3.5 | 3 | 2 | LTF | ||
| 25 | 18 | M | Unknown | 7 | DOD | 15.8 years | ||
| 26 | 19 | F | 1.5 | 4 | 1 | ANED | 17.5 years | |
| 27 | 19 | F | 10 | 1 | 3a | Liver metastasis, 42 years later | AWD | 44 years |
| 28 | 19 | F | 12 | 3 | 3b | Lymph node metastasis at presentation | ANED | 16.6 years |
| 29 | 19 | F | 4.5, 2.5 and 1.5 cm (three) | 11 | 5 | Received imatinib for 5 years | ANED | 7.0 years |
| 30 | 21 | F | 7 | 13 | 6a | ANED | 10.1 years | |
| 31 | 21 | M | 7 plus smaller ones | 2 | 3a | ANED | 17.3 years | |
| 32 | 21 | F | Unknown | 16 | * | Peritoneal metastases at presentation | LTF | |
| 33 | 21 | F | 11 (rec) | Gastric recurrence, 9 years | AWD | 9 years | ||
| 34 | 22 | F | 5 | 9 | 5 | LTF | ||
| 35 | 22 | F | 5 + smaller nodule | 15 | 5 | Gastric recurrence and peritoneal metastases 22.7 years later. Total gastrectomy. | ANED | 33 years |
| 36 | 24 | F | 2.2 | 0 | 2 | LTF | ||
| 37 | 25 | F | 4 | 5 | 2 | ANED | 8.5 years | |
| 38 | 25 | F | Unknown | 11 | * | LTF | ||
| 39 | 26 | F | 5.8 | 14 | 6a | Liver and lymph node metastasis at presentation | DOD | 3.3 years |
| 40 | 28 | F | Large palpable tumor, multiple gastric nodules | 1 | * | Gastric ulcer diagnosis six years before the GIST | ANED | 31.5 years |
| 41 | 29 | F | 5 | 9 | 5 | ANED | 24.7 years | |
| 42 | 29 | F | 5 + 4.5 cm and two smaller nodules | 14 | 5 | Lymph node metastases and omental nodules at presentation | ANED | 9 years |
| 43 | 29 | M | 10 | 7 | 6a | Gastric recurrence, 5 years and carotid body paraganglioma 25.5 years | ANED | 25.5 years |
| 44 | 30 | M | 7 | 11 | 6a | Recurrence in abdominal soft tissue at 12.8 years | AWD | 14.7 years |
| 45 | 30 | M | 6.1 + 3.2 cm | 11 | 3a | ANED | 7.2 years | |
| 46 | 31 | F | 5 cm, plus smaller separate nodules | 11 | 6a | ANED | 16.3 years | |
| 47 | 32 | F | 6.5 | 30 | 6a | Metastases, not further specified | DOD | 1.3 years |
| 48 | 33 | M | 5 | 2 | ANED | 2.5 years | ||
| 49 | 35 | F | ND | 2 (rec) | * | Liver metastasis, unknown interval | LTF | |
| 50 | 37 | F | 3 | 1 | 2 | ANED | 10.3 years | |
| 51 | 37 | F | 5 | 2 | 2 | LTF | ||
| 52 | 37 | M | 5 | 0 | 2 | DUC | 21.0 years | |
| 53 | 38 | F | 9 | 4 | 3a | Lymph node metastasis at presentation | ATSU | 3.6 years |
| 54 | 39 | F | 3 | 5 | 2 | ANED | 9.9 years | |
| 55 | 39 | M | 6.5 | 4 | 3a | Pheochromocytoma 24 years before GIST (at age of 15 years) | ANED | 5.8 years |
| 56 | 39 | M | ND | 3 | LTF | |||
| 57 | 40 | F | 4.5 | 3 | 2 | ANED | 18.8 years | |
| 58 | 48 | M | ND | 1 | LTF | |||
| 59 | 51 | M | 4 | 1 | 2 | ANED | 16.8 years | |
| 60 | 52 | M | 10 | 3 | 3a | LTF | ||
| 61 | 56 | F | >10, multinodular | 1 | 3b | ANED | 31.0 years | |
| 62 | 61 | M | 1.5 | 3 | 2 | ANED | 9.5 years | |
| 63 | 61 | M | Large, liver metastasis with rupture | Primary tumor not studied | * | DOD | 2.2 years | |
| 64 | 68 | M | 2.5 | 0 | 2 | DOPC | ||
| 65 | 71 | F | 5.5 | 4 | 3a | ANED | 11.0 years | |
| 66 | 77 | F | 3.5 | 0 | 2 | DUC | 14.7 years |
*
Could not be assessed. rec = based on evaluation of recurrent tumor.