Department of Surgery, Kuma Hospital, Kobe, Japan.
Ishikawa Gastroenterology Clinic, Osaka, Japan.
Thyroid. 2019 Oct;29(10):1511-1517. doi: 10.1089/thy.2019.0121. Epub 2019 Sep 25.
The cribriform-morular variant (CMV) is a rare subtype of papillary thyroid carcinoma (PTC), and is often associated with familial adenomatous polyposis (FAP). This variant is generally indolent, but some aggressive cases have been reported. We present the case of a 24-year-old woman who underwent total thyroidectomy with prophylactic central lymph node dissection and modified radical neck dissection for CMV-PTC. No distant metastases were identified preoperatively. However, multiple large lung metastases were detected three and half years after surgery. She also had FAP with a germline gene mutation. She was started on lenvatinib because of the metastatic disease. One month after the initiation of lenvatinib (24 mg), her lung metastases reduced significantly. She has continued lenvatinib for 24 months (present dose, 10 mg). The lung metastases have not progressed during this period. Only a few small polyps were newly detected on endoscopy after lenvatinib administration. This number was considerably higher before therapy, when an average of 21-75 polyps were resected at each endoscopy session. This is the first report of the use of lenvatinib for the treatment of distant metastases from CMV-PTC. In addition to the efficacy in treating metastatic lesions of this rare variant of PTC, lenvatinib shows promise in the management of associated FAP. This treatment strategy may be useful in the management of recurrent CMV-PTC and in those with FAP who refuse colectomy.
筛状-微乳头变异型(CMV)是甲状腺乳头状癌(PTC)的一种罕见亚型,常与家族性腺瘤性息肉病(FAP)相关。这种变异型通常是惰性的,但也有报道一些侵袭性病例。我们报告了一例 24 岁女性患者,因 CMV-PTC 行全甲状腺切除术加预防性中央淋巴结清扫术和改良根治性颈淋巴结清扫术。术前未发现远处转移。然而,术后 3 年半时发现多个大的肺转移灶。该患者还患有 FAP,存在种系 基因突变。由于转移性疾病,她开始接受仑伐替尼治疗。仑伐替尼(24mg)起始治疗 1 个月后,肺部转移灶明显缩小。此后,她继续接受仑伐替尼治疗 24 个月(目前剂量为 10mg)。在此期间,肺部转移灶未进展。仑伐替尼治疗后,仅在内镜下新发现几个小息肉。在治疗前,每次内镜检查平均切除 21-75 个息肉,数量明显更高。这是首例使用仑伐替尼治疗 CMV-PTC 远处转移的报告。除了对这种罕见 PTC 变异型转移灶的疗效外,仑伐替尼在 FAP 的管理中也显示出前景。这种治疗策略可能对复发性 CMV-PTC 的管理以及拒绝结肠切除术的 FAP 患者有用。