背景:甲状旁腺癌非常罕见,代表<1%的原发性甲状旁腺功能亢进病例。方法:回顾性分析2001年至2018年间接受甲状旁腺疾病评估的患者资料。目的是描述临床表现,组织病理学特征,和甲状旁腺癌的治疗结果。结果:我们从多伦多四级护理内分泌肿瘤学的门诊实践中确定了8例甲状旁腺癌,加拿大。临床表现如下:5/8例(62.5%)症状性高钙血症和3/8例(37.5%)可疑甲状腺结节。在所有7例术前钙测量中,高钙血症均明显。组织病理学特征包括:7/8例(87.5%)的血管浸润和纤维旁蛋白的免疫组织化学丢失,视网膜母细胞瘤,或p27在所有8种情况下。额外治疗包括:5/8例(62.5%)外照射放疗,2/8患者化疗(25%),和3/8的额外手术(37.5%)。只有2例患者(25%)在手术治疗后长期缓解,其他人有持续性(3例)或复发性疾病(3例)。五名患者发展为转移性疾病,都涉及肺。在接受索拉非尼治疗的两名患者中,有证据表明肺转移消退。一名患者死于疾病进展。结论:在这一系列甲状旁腺癌患者中,主要表现为有症状的高钙血症和血管浸润性疾病,只有少数人获得了持久的缓解。肺是远处转移的最常见部位。手术导致两例缓解,但6例持续性或复发性疾病患者均未最终实现疾病缓解。
Background: Parathyroid carcinoma is rare, representing <1% of primary hyperparathyroidism cases. Methods: Retrospective data of patients referred for evaluation of parathyroid disease between 2001 and 2018 were reviewed. The goal was to describe the clinical presentation, histopathologic characteristics, and treatment outcomes of parathyroid carcinoma. Results: We identified 8 cases of parathyroid carcinoma from the outpatient practice of a quaternary care Endocrine Oncology practice in Toronto, Canada. The clinical presentation was as follows: 5/8 cases (62.5%) of symptomatic hypercalcemia and 3/8 cases (37.5%) of a suspicious thyroid nodule. Hypercalcemia was evident in all 7 cases with pre-operative calcium measurements. Histopathologic features included: vascular invasion in 7/8 cases (87.5%) and immunohistochemical loss of either parafibromin, retinoblastoma, or p27 in all 8 cases. Additional treatment included: external beam radiotherapy in 5/8 cases (62.5%), chemotherapy for 2/8 patients (25%), and additional surgery for 3/8 patients (37.5%). Only 2 patients (25%) had long-term remission following surgical treatment, and the others had either persistent (3 patients) or recurrent disease (3 patients). Five patients developed metastatic disease, all involving lung. In one of two patients treated with Sorafenib there was evidence of regression of lung metastases. One patient died of disease progression. Conclusion: In this series of patients with parathyroid carcinoma largely presenting with symptomatic hypercalcemia and angioinvasive disease, only a minority achieved a durable remission. Lung was the most common site of distant metastasis. Surgery led to remission in two cases, but none of the six patients with persistent or recurrent disease ultimately achieved disease remission.