Parathyroid cancer (PC) is a rare malignant tumor which comprises 0.5-5% of patients with primary hyperparathyroidism (PHPT). Most of these cancers are sporadic, although it may also occur as a feature of various genetic syndromes including hyperparathyroidism-jaw tumor syndrome (HPT-JT) and multiple endocrine neoplasia (MEN) types 1 and 2A. Although PC is characterized by high levels of serum ionized calcium (Ca) and parathyroid hormone (PTH), the challenge to the clinician is to distinguish PC from the far more common entities of parathyroid adenoma (PA) or hyperplasia, as there are no specific clinical, biochemical, or radiological characteristic of PC. Complete surgical resection is the only known curative treatment for PC with the surgical approach during initial surgery strongly influencing the outcome. In order to avoid local recurrence, the lesion must be removed en-bloc with clear margins. PC has high recurrence rates of up to 50% but with favorable long-term survival rates (10-year overall survival of 60-70%) due to its slow-growing nature. Most patients die not from tumor burden directly but from uncontrolled severe hypercalcemia. In this article we have updated the information on PC by reviewing the literature over the past 10 years and summarizing the findings of the largest series published in this period.

中文翻译：

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甲状旁腺癌：更新。

甲状旁腺癌（PC）是一种罕见的恶性肿瘤，占原发性甲状旁腺功能亢进症（PHPT）患者的0.5-5％。这些癌症大多数都是散发性的，尽管它也可能是多种遗传综合症的特征，包括甲状旁腺功能亢进-下颌肿瘤综合症（HPT-JT）和多发性内分泌肿瘤（MEN）1型和2A型。尽管PC的特征在于高水平的血清离子钙（Ca）和甲状旁腺激素（PTH），但临床医生面临的挑战是将PC与更常见的甲状旁腺腺瘤（PA）或增生实体区分开来，因为没有特异性PC的临床，生化或放射学特征。完全手术切除是唯一已知的PC根治性治疗，其在初次手术期间会通过手术方式严重影响预后。为了避免局部复发，病变必须整体清除，边缘清楚。PC的复发率高达50％，但由于其生长缓慢，具有良好的长期生存率（10年总生存率60-70％）。大多数患者不是直接由于肿瘤负担而死亡，而是由于无法控制的严重高钙血症而死亡。在本文中，我们通过回顾过去10年的文献并总结了这一时期出版的最大系列的研究成果，来更新了PC上的信息。