Quinn Courtney E, Healy James, Lebastchi Amir H, Brown Taylor C, Stein Juliana E, Prasad Manju L, Callender Glenda G, Carling Tobias, Udelsman Robert
Department of Surgery, Section of Endocrine Surgery, Yale University School of Medicine, New Haven, CT.
Department of Surgery, Section of Endocrine Surgery, Yale University School of Medicine, New Haven, CT.
J Am Coll Surg. 2015 Jun;220(6):1054-62. doi: 10.1016/j.jamcollsurg.2014.10.007. Epub 2014 Oct 24.
Parathyroid carcinoma (PTCA) is an exceptionally rare malignancy, often with a clinical presentation similar to that of benign atypical parathyroid adenoma. Its low incidence portends unclear guidelines for management. Accordingly, thorough examination of clinical and pathologic variables was undertaken to distinguish between PTCA and atypical adenomas.
This was a retrospective analysis of a prospective database at a tertiary academic referral center. Between September 2001 and April 2014, 3,643 patients were referred for surgical treatment of PHPT. Of these, 52 harbored aggressive parathyroid tumors: parathyroid carcinomas (n=18) and atypical adenomas (n=34). We analyzed the surgical and clinicopathologic tumor characteristics, and did a statistical analysis. We measured preoperative and intraoperative variables, and postoperative and pathologic outcomes.
Parathyroid carcinoma patients present with significantly increased tumor size (3.5 cm vs 2.4 cm, respectively; p=0.002), mean serum calcium (13.0 vs 11.8 mg/dL, respectively; p=0.003) and intact parathyroid hormone (iPTH) levels (489 vs 266 pg/mL, respectively; p=0.04), and a higher incidence of hypercalcemic crisis, compared with patients with atypical adenomas (50% vs 19%, respectively; p=0.072). Parathyroid carcinoma more frequently lacks a distinct capsule (47.1% vs 12.9%, respectively; p=0.03) and adheres to adjacent structures (77.8% vs 20.6%, respectively; p=0.017). Of note, there was no significant difference in loss of parafibromin expression between groups.
Clinical distinction between PTCA and atypical adenomas is of critical importance in determining the appropriate extent of resection and follow-up. Loss of parafibromin has not been shown to distinguish between PTCA and atypical adenoma; clearer definition of clinicopathologic criteria for PTCA is warranted and may lead to improved postoperative management.
甲状旁腺癌（PTCA）是一种极其罕见的恶性肿瘤，其临床表现通常与良性非典型甲状旁腺腺瘤相似。由于其发病率低，管理指南尚不明确。因此，我们对临床和病理变量进行了全面检查，以区分PTCA和非典型腺瘤。
这是一项对三级学术转诊中心前瞻性数据库的回顾性分析。2001年9月至2014年4月期间，3643例患者因原发性甲状旁腺功能亢进症（PHPT）接受手术治疗。其中，52例患有侵袭性甲状旁腺肿瘤：甲状旁腺癌（n = 18）和非典型腺瘤（n = 34）。我们分析了手术及临床病理肿瘤特征，并进行了统计分析。我们测量了术前和术中变量，以及术后和病理结果。
与非典型腺瘤患者相比，甲状旁腺癌患者的肿瘤大小显著增加（分别为3.5 cm和2.4 cm；p = 0.002），平均血清钙水平（分别为13.0和11.8 mg/dL；p = 0.003）和完整甲状旁腺激素（iPTH）水平（分别为489和266 pg/mL；p = 0.04），高钙血症危象的发生率更高（分别为50%和19%；p = 0.072）。甲状旁腺癌更常缺乏完整的包膜（分别为47.1%和12.9%；p = 0.03）并侵犯相邻结构（分别为77.8%和20.6%；p = 0.017）。值得注意的是，两组之间副纤维蛋白表达缺失无显著差异。
PTCA与非典型腺瘤的临床鉴别对于确定合适的切除范围和随访至关重要。副纤维蛋白缺失尚未被证明可区分PTCA和非典型腺瘤；有必要更明确地定义PTCA的临床病理标准，这可能会改善术后管理。
