Alsanea O
Endocine Surgical Unit, University of California, San Francisco/Mount Zion Medical Center, 94143-1674, USA.
Curr Treat Options Oncol. 2000 Oct;1(4):345-51. doi: 10.1007/s11864-000-0050-9.
Familial nonmedullary thyroid cancer is more aggressive than sporadic nonmedullary thyroid cancer. It tends to affect younger patients, and the tumors are often multi-focal and bilateral. Histologically, 90% of these tumors are papillary cancers and the remaining are Hürthle cell cancers. We recommend total thyroidectomy to remove all the thyroid tissue, which harbors the genetic defect responsible for the disease (even in low-risk patients) due to the predisposition to develop thyroid cancer and the more aggressive nature of the disease. Careful exploration of the ipsilateral lymph nodes with ipsilateral central neck dissection is encouraged to decrease a high recurrence rate (44%). A complete modified radical neck dissection should be limited to a therapeutic role because there is no clear evidence that this procedure carries any survival benefit. We also recommend that patients receive radioactive iodine ablation post-operatively, including a prophylactic dose (30 mCi) for patients with no evidence of residual uptake on the postoperative iodine 131 whole body scan and in low-risk patients using any of the prognostic scoring systems. Patients should be placed on enough thyroid hormone to suppress thyroid-stimulating hormone (TSH) to approximately 0.1 mL/mL in low-risk patients and to less than 0.1 mL/mL in high-risk patients. Focal metastatic disease in patients with familial nonmedullary thyroid cancer is best dealt with by surgical excision followed by radioactive iodine ablation when appropriate. Redifferentiation therapy has a promising role in patients who have radioactive iodine-resistant tumors. The value of prevention, early detection, and targeted gene therapy once the gene or genes responsible for familial non-medullary thyroid cancer have been identified cannot be overemphasized.
家族性非髓样甲状腺癌比散发性非髓样甲状腺癌更具侵袭性。它往往影响较年轻的患者，肿瘤通常为多灶性和双侧性。组织学上，这些肿瘤90%为乳头状癌，其余为许特莱细胞癌。由于易患甲状腺癌以及该疾病更具侵袭性的特点，我们建议行全甲状腺切除术以切除所有甲状腺组织，该组织中存在导致该病的基因缺陷（即使是低风险患者）。鼓励仔细探查同侧淋巴结并进行同侧中央区颈清扫，以降低高复发率（44%）。完全改良根治性颈清扫应仅限于治疗作用，因为没有明确证据表明该手术有任何生存获益。我们还建议患者术后接受放射性碘消融，对于术后碘131全身扫描无残留摄取证据的患者以及使用任何预后评分系统的低风险患者，给予预防性剂量（30 mCi）。低风险患者应补充足够的甲状腺激素以将促甲状腺激素（TSH）抑制至约0.1 mIU/mL，高风险患者抑制至低于0.1 mIU/mL。家族性非髓样甲状腺癌患者的局限性转移病灶最好通过手术切除，然后在适当时进行放射性碘消融。再分化疗法在对放射性碘耐药的肿瘤患者中具有广阔前景。一旦确定了导致家族性非髓样甲状腺癌的一个或多个基因，预防、早期检测和靶向基因治疗的价值再怎么强调也不为过。
