Liu Ruifeng, Ma Liyuan, Xia Yu, Gao Luying, Ji Jiang, An Yuang, Pan Aonan, Luo Nengwen, Jiang Yuxin
Department of Ultrasound, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Department of Ultrasound, Peking University Shenzhen Hospital, Shenzhen, China.
Orphanet J Rare Dis. 2025 Mar 4;20(1):100. doi: 10.1186/s13023-025-03608-4.
To investigate prognostic factors related with parathyroid carcinoma (PC) based upon ultrasound (US) parameters and parafibromin expression.
Between 2000/01 and 2022/07, thirty-four PC patients with detailed preoperative ultrasonography were enrolled in the research. Immunohistochemical staining of parafibromin was performed on pathological samples of these patients. Based on the expression of parafibromin, the cases were divided into a positive control group (parafibromin expression ≥ 10%) and a negative experimental group (parafibromin expression < 10%). The ultrasound and clinical features of the two groups were analyzed, and Cox regression was used to identify the independent prognostic factors regarding disease-free survival (DFS) and overall survival (OS).
Among 34 patients with parathyroid carcinoma, 26 (76.5%) were parafibromin-positive, while 8 (23.5%) were parafibromin-negative. The mean follow-up time was 72.6 (11.0-179.3) months. During the overall survival period, 7 cases (20.6%) died, and 9 cases (26.5%) experienced recurrence or metastasis. The median overall survival time (interquartile range) was 65.7 (35.5-89.7) months, and the median disease-free survival time (interquartile range) was 38.2 (22.2-69.7) months. The risk of recurrence and metastasis in the parafibromin-negative group was 5.9 times higher than that in parafibromin-positive group (95% CI 1.569-22.190). PC patients with calcification on preoperative ultrasonography had a 9.4 times higher risk of death during the overall survival period compared with patients without calcification (95% CI 1.037-85.915). However, parafibromin expression did not show a significant impact on the prognosis of the overall survival.
Preoperative US-detected calcification within the lesion is an independent risk factor indicating the shorter OS for PC patients, while loss of parafibromin expression is significant for indicating the recurrence or metastasis of PC patients.
基于超声（US）参数和 parafibromin 表达情况，研究与甲状旁腺癌（PC）相关的预后因素。
在 2000 年 1 月至 2022 年 7 月期间，34 例术前行详细超声检查的 PC 患者被纳入研究。对这些患者的病理样本进行 parafibromin 的免疫组织化学染色。根据 parafibromin 的表达情况，将病例分为阳性对照组（parafibromin 表达≥10%）和阴性试验组（parafibromin 表达<10%）。分析两组的超声及临床特征，并采用 Cox 回归分析确定无病生存期（DFS）和总生存期（OS）的独立预后因素。
34 例甲状旁腺癌患者中，26 例（76.5%）parafibromin 呈阳性，8 例（23.5%）parafibromin 呈阴性。平均随访时间为 72.6（11.0 - 179.3）个月。在总生存期内，7 例（20.6%）死亡，9 例（26.5%）出现复发或转移。总生存期的中位时间（四分位间距）为 65.7（35.5 - 89.7）个月，无病生存期的中位时间（四分位间距）为 38.2（22.2 - 69.7）个月。parafibromin 阴性组复发和转移的风险比阳性组高 5.9 倍（95%CI 1.569 - 22.190）。术前超声检查有钙化的 PC 患者在总生存期内死亡风险比无钙化患者高 9.4 倍（95%CI 1.037 - 85.915）。然而，parafibromin 表达对总生存期的预后未显示出显著影响。
术前 US 检测到病变内有钙化是提示 PC 患者总生存期较短的独立危险因素，而 parafibromin 表达缺失对提示 PC 患者复发或转移具有重要意义。
