Fondazione FIRMO Onlus, Italian Foundation for the Research on Bone Diseases, 50129 Florence, Italy.
Bone Metabolic Diseases Unit, Department of Experimental and Clinical Biomedical Sciences, University of Florence, University Hospital of Florence, 50134 Florence, Italy.
Eur J Endocrinol. 2024 Sep 30;191(4):416-425. doi: 10.1093/ejendo/lvae121.
Atypical parathyroid tumor (aPT) and parathyroid carcinoma (PC) are extremely rare parathyroid neoplasms, accounting together for <2% of all parathyroid tumors. They often present an overlapping clinical phenotype, sharing clinical, biochemical, and some histological features. They are distinguished only by the presence of local invasion, and lymph nodes or distant metastasis, which are all absent in aPTs. To date, only few studies have compared clinical presentation and features between aPTs and PCs. Our purpose was to conduct a retrospective study on a multicenter Italian database of aPT and PC patients.
We comparatively analyzed main features of aPT (n = 57) and PC (n = 74) patients collected at 15 major endocrinology and endocrine surgery centers in Italy.
Atypical parathyroid tumors and PCs showed no significant differences in many clinical features and presented similar values of elevated parathyroid hormone and total serum calcium. Renal complications, namely nephrolithiasis and nephrocalcinosis, appeared to be more common in PC, with a significantly higher rate of renal colic, regardless of total serum calcium levels and 24-h calciuria. Parathyroid carcinomas showed significantly higher postoperative disease persistence and recurrence rates, presumably due to an uncomplete resection of the primary tumor in 23.5% of cases and/or presence of unremoved active metastasis, but they had similar disease-free mean time after surgery than aPT. To deepen the study of malignant parathyroid tumors, the institution of a novel Italian retro-prospective multicenter registry of aPTs and PCs is currently ongoing, and a dedicated PC European registry has been recently activated.
非典型甲状旁腺肿瘤（aPT）和甲状旁腺癌（PC）是极为罕见的甲状旁腺肿瘤，占所有甲状旁腺肿瘤的比例<2%。它们常表现出重叠的临床表型，具有相似的临床、生化和一些组织学特征。只有局部侵犯、淋巴结或远处转移才能将它们区分开来，这些在 aPT 中均不存在。迄今为止，仅有少数研究比较了 aPT 和 PC 患者的临床表现和特征。我们的目的是对意大利 15 个主要内分泌学和内分泌外科中心的 aPT 和 PC 患者的多中心意大利数据库进行回顾性研究。
我们比较分析了在意大利 15 个主要内分泌学和内分泌外科中心收集的 57 例 aPT 和 74 例 PC 患者的主要特征。
aPT 和 PC 在许多临床特征上无显著差异，且甲状旁腺激素和总血清钙升高值相似。肾结石和肾钙质沉着症似乎在 PC 中更为常见，肾绞痛发生率显著更高，而与总血清钙水平和 24 小时尿钙无关。PC 的术后疾病持续存在和复发率显著更高，可能是由于 23.5%的病例未完全切除原发肿瘤和/或存在未切除的活性转移，但它们的无疾病中位时间与 aPT 相似。为了深入研究恶性甲状旁腺肿瘤，目前正在进行一项新的意大利回顾性多中心 aPT 和 PC 登记研究，并且最近已经启动了一项专门的 PC 欧洲登记研究。
