This work is licensed under a Table 2.
Review of patients with metastatic pheochromocytoma/paraganglioma in MEN2 syndrome.
| Case | Age (year)/gender (M/F) | Syndromic features | Genetics (RET Codon) | Pheochromocytoma/paraganglioma | Metastasis | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| MTC (Y/N) | TTx (Y/N)/CTE (Y/N) | PHPT (Y/N) | HTN (Y/N) | Biochemistry (×UNL) | Primary site | Size (cm) | Metastatic site | Chronousity (SC/MC) | Adrenal surgery (Y/N)/CE (Y/N) | Modality for localization | HPR (Y/N) | Treatment | Follow-up (month) | Outcome at last FU death (Y/N) | |||
| 1. PFMN, 2. PFNMN, 3. UNMN, 4. UMN, 5. UE, 6. UNE | 1. MIBG, 2. FDG, 3. DOTA, 4. CT/MRI, 5. Intra-OP, 6. Autopsy, 7. SVS, 8. DOPA | ||||||||||||||||
| 1. (10) | 18/F | Y | Y | Y | – | B/L | 5 | – | – | – | – | – | – | 384 | N | ||
| 2. (10) | 28/F | Y | Y | Y | – | B/L | 12 | – | – | – | – | – | – | 384 | N | ||
| 3. (10) | 20/F | Y | Y | N | 4: 27.9 | B/L | 13 | – | – | – | – | – | – | – | Y | ||
| 4. (10) | 23/F | Y | Y | Y | – | B/L | 12 | – | – | – | – | – | – | – | Y | ||
| 5. (11) | 53/F | – | – | B/L | 12 | Liver | – | – | – | – | – | – | – | ||||
| 6. (11) | 38/F | – | – | B/L | – | Liver | – | – | – | – | – | – | – | ||||
| 7. (12) | 49/M | Y | N/– | – | – | B/L | 19 | Lung, heart | SC | N/– | 6: Lung, heart | Y | – | 0 | Y | ||
| 8. (13) | 20/F | Y | Y/– | Y | B/L | 11 | Liver, spleen, pancreas |
SC | N/– | 1: Liver, 4: Liver 5: Liver, spleen, pancreas |
– | – | – | – | |||
| 9. (14) | 44/F | Y | Y/N | – | – | B/L | – | Lung | MC | Y/Y | 1: Lung, 5: Lung | – | Surgery | – | N | ||
| 10. (15) | 26/M | Y | Y | 5: 5, 6: 63.4 | Left | – | – | – | – | – | – | – | – | N | |||
| 11. (15) | 40/F | Y | Y | – | B/L | – | – | – | – | – | – | – | – | Y | |||
| 12. (16) | 23/M | Y | N/Y | Y | 5: 7.8, 6: 4.5 | Left | – | Lung, liver | SC | Y/Y | 1: Lung, liver | MIBG therapy | 84 | N | |||
| 13. (17) | 35/M | Y | N | 3: 1.63 | B/L | 6 | Lymph node | – | – | – | Y | – | – | – | |||
| 14. (18) | 39/F | Y | N/Y | Y | 3: 29.8, 4: 8.3 5: 1.2, 6: 1.5 |
B/L | – | Lung, livera, bone |
MC | Y/Y | 1: Lung, liver, 4: Liver, 7: Liver |
Y | MIBG therapy, Chemotherapy |
48 | Y | ||
| 15. (19) | 28/M | Y | Y/Y | Y | 5: 21, 6: 4.0 | B/L | 12 | Bone | MC | Y/Y | 1: Bone, 4: Bone | – | MIBG therapy | 12 | N | ||
| 16. (20) | 53/M | Y | Y/ | Y | 634 | – | B/L | 9.4 | Lung, liver | SC | Y/– | – | Y | Angioembolisation | 2 | Y | |
| 17. (21) | 31/F | Y | Y/ | Y | – | Left | – | Brain | MC | Y/ | 4: Brain | Y | Surgery | 48 | N | ||
| 18. (22) | 47/M | Y | N/Y | 634 | Y | – | B/L | 7 | Bone, liver | SC | N/Y | 1: Bone, 4: Bone | Y | MIBG therapy, RT | 24 | N | |
| 19. (23) | 65/M | Y | N/Y | 5: 5.25, 6: 1.2 | B/L | 8 | Liver | MC | Y/Y | – | Y | – | – | Y | |||
| 20. (24) | 34/F | Y | N/Y | Y | 634 | Y | 4: >1 | B/L | – | Pancreas | SC | N/Y | 5: Pancreas | Y | Surgery | 6 | N |
| 21. (25) | 21/F | Y | N/Y | N | 634 | Y | 3: 104, 4:99.6 | Right | 12 | Heart | SC | N/Y | 4: Heart | Y | Surgery | 5 | N |
| 22. (26) | 41/M | N | N/– | – | – | – | B/L | 4 | Skin, lung | SC | N/– | – | Y | – | – | Y | |
| 23. (27) | 65/M | 804 | – | – | U/L | 9.5 | – | – |
– | – | – | – | – | – | |||
| 24 (28) | 47/F | 634 | – | – | Right | 10 | Bone | MC | Y/– | 1: Bone, 8: Bone | – | – | – | – | |||
| 25. (29) | 39/F | 791 | – | – | U/L | 10 | Lymph node | MC | – | – | – | –– | – | – | |||
| 26. (30) | 55/F | N | N/N | Y | 531 | Y | 3: 2.6, 4:0.9 | B/L | – | Liver, lung | MC | Y/– | 1: Liver, lung | – | MIBG therapy | 96 | N |
| 27. (31) | 48/F | Y | N/Y | N | 634 | Y | 3: 7.6, 4:18.3 | B/L | 4.1 | Liver | MC | Y/Y | 2: Liver, 3: Liver, 4: Liver | Y | Chemotherapy | 4 | N |
| 28. (32) | 25/F | 634 | – | – | B/L | 8 | – | – | – | – | – | – | – | – | |||
| 29. (33) | 19/M | Y | N/Y | N | 918 | – | 1: 12, 3:15.9 4: 14.8 |
Right | 11 | Bone, lung | SC | N/Y | 1: Bones, lung, 4: Bones, lung |
Y | Chemotherapy, sunitinib | 0 | Y |
| 30. Case A | 54/F | Y | N/Y | N | 618 | N | 1: 20 2: 18.3 |
Right | 6.1 | Bone | SC | Y/Y | 1: Bones, 3: Bones, 4: Bones | Y | MIBG therapy, angio-embolisation | 6 | N |
| 31. Case B | 45/F | Y | Y/N | Y | 634 | Y | 2: 16.9 | B/L | 8.3 | Liver | MC | Y/Y | 2: Liver, 5: Liver | Y | – | 0 | Y |
aDiagnosed by selective venous sampling of hepatic vein and inferior vena cava.
Adx, adrenal surgery; B/L, bilateral; CE, catecholamine excess; CTE, calcitonin excess; CVD, cyclophosphamide, vincristine, dacarbazine; DOTA, 68Ga-DOTATATE PET CT scan; F, female, FDG, 18fluorodeoxyglucose PET CT scan; FU, follow-up; HA, hepatic artery; HPR, histopathological report; HTN, hypertension; Intra-OP, intraoperatively; M, male; MC, metachoronous; MTC, medullary thyroid carcinoma; MIBG, 131metaiodobenzylguanidine; N, no; PFMN, plasma-free metanephrins; PFNMN, plasma-free normetanephrins; PHPT, primary hyperparathyroidism; RT, radiotherapy; SC, synchoronous; TTx, total thyroidectomy; U/L, unilateral; UNMN, 24 h urinary normetanephrines; UMN, 24 h urinary metanephrins; UE, 24 h urinary epinephrines; UNE, 24 h urinary norepinephrines; ×UNL, times the upper normal limit; Y, yes; –, data not available.