Collini Paola, Mattavelli Franco, Spinelli Claudio, Massimino Maura
Istituto Nazionale Tumori, Anatomic Pathology C Unit, Via Venezian, 1, Milan, Italy.
Expert Rev Anticancer Ther. 2007 Jan;7(1):23-30. doi: 10.1586/14737140.7.1.23.
Nonmedullary thyroid carcinomas are rare malignancies in pediatric ages. The vast majority of them are papillary carcinomas with an overall survival of approximately 100%. Their outcome is independent of strong prognostic factors of adults, such as papillary carcinoma histological subtype, invasion into soft tissue of the neck, presence and site of distant metastases, relapse and type of surgery. In these ages, follicular carcinomas and poorly differentiated carcinomas are exceptional. Undifferentiated (anaplastic) carcinomas are practically absent. In most institutions, the therapy of choice for all pediatric thyroid carcinomas is the radical approach, aimed at the eradication at diagnosis of all clinical and subclinical neoplastic foci, both at thyroid, lymph node and distant level. It consists of total thyroidectomy and lymphadenectomy in children with clinically evident lymph-node metastases, followed by radioactive iodine therapy independent of histotype and stage. Recently, owing also to the high sensitivity to hormonal manipulation shown by pediatric papillary carcinomas, a conservative approach has been proposed for selected cases, consisting of the removal of only the grossly detectable disease followed by thyroid-stimulating hormone-suppressive hormonal therapy to control subclinical disease. Today, the existence of two therapeutic approaches, radical versus conservative therapy, should be considered whenever treating a child or adolescent with a nonmedullary thyroid carcinoma. Not least, permanent post-treatment complications of radical surgery and radioactive iodine therapy should be taken into account. The future tasks include the stratification of thyroid carcinomas into low- and high-risk cases, also including their molecular alterations and the possibility of a molecularly targeted therapy against tyrosine kinases involved in the pathogenesis of thyroid carcinomas.
非髓样甲状腺癌在儿童期是罕见的恶性肿瘤。其中绝大多数是乳头状癌,总体生存率约为100%。其预后与成人的强预后因素无关,如乳头状癌的组织学亚型、侵犯颈部软组织、远处转移的存在和部位、复发及手术类型。在这些年龄段,滤泡状癌和低分化癌较为罕见。未分化(间变性)癌实际上不存在。在大多数机构中,所有儿童甲状腺癌的首选治疗方法是根治性方法,旨在在诊断时根除甲状腺、淋巴结和远处的所有临床和亚临床肿瘤病灶。对于有临床明显淋巴结转移的儿童,治疗包括全甲状腺切除术和淋巴结清扫术,随后进行放射性碘治疗,与组织学类型和分期无关。最近,由于儿童乳头状癌对激素操纵显示出高敏感性,对于某些选定病例提出了一种保守方法,即仅切除肉眼可见的病灶,随后进行促甲状腺激素抑制性激素治疗以控制亚临床疾病。如今,在治疗儿童或青少年非髓样甲状腺癌时,应考虑存在根治性与保守性两种治疗方法。尤其要考虑根治性手术和放射性碘治疗的永久性治疗后并发症。未来的任务包括将甲状腺癌分层为低风险和高风险病例,还包括其分子改变以及针对参与甲状腺癌发病机制的酪氨酸激酶进行分子靶向治疗的可能性。