非典型甲状旁腺肿瘤（aPT）和甲状旁腺癌（PC）是极其罕见的甲状旁腺肿瘤，合计占所有甲状旁腺肿瘤的<2%。它们通常呈现重叠的临床表型，具有共同的临床、生化和一些组织学特征。它们的区别仅在于存在局部侵袭、淋巴结或远处转移，而 aPT 中均不存在这些情况。迄今为止，只有少数研究比较了 aPT 和 PC 的临床表现和特征。我们的目的是对意大利多中心aPT和PC患者数据库进行回顾性研究。我们比较分析了意大利15个主要内分泌科和内分泌手术中心收集的aPT（n = 57）和PC（n = 74）患者的主要特征非典型甲状旁腺肿瘤和PC在许多临床特征上没有显着差异，并且甲状旁腺激素和总血清钙的升高值相似。肾脏并发症，即肾结石和肾钙质沉着症，在 PC 中似乎更常见，无论血清总钙水平和 24 小时尿钙水平如何，肾绞痛的发生率显着较高。甲状旁腺癌术后疾病持续率和复发率显着较高，可能是由于 23.5% 的病例中原发肿瘤未完全切除和/或存在未切除的活动性转移灶，但术后平均无病时间与 aPT 相似。为了深化恶性甲状旁腺肿瘤的研究，目前正在建立一个新颖的意大利回顾性多中心 aPT 和 PC 登记处，并且最近启动了一个专门的 PC 欧洲登记处。© 作者 2024。由牛津出版大学出版社代表欧洲内分泌学会。版权所有。如需商业重复使用，请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限都可以通过我们网站文章页面上的权限链接通过我们的 RightsLink 服务获得 - 如需了解更多信息，请联系journals.permissions@oup.com。

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.© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.