Division of Endocrinology, Metabolism and Bone and Mineral Disorders, Henry Ford Health, Detroit, MI, United States.
Department of Endocrinology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Front Endocrinol (Lausanne). 2023 Feb 2;14:1009516. doi: 10.3389/fendo.2023.1009516. eCollection 2023.
Parathyroid adenomas weighing more than 3.5 g are reported variously as "atypical", "large" or "giant" parathyroid adenomas. All such adenomas are rare variants accounting for no more than 1.5% of all parathyroid adenomas. Large parathyroid adenomas are often associated with more severe form of the disease, including osteitis fibrosa cystica (OFC) and share many biochemical, histological, and molecular features of both benign and malignant parathyroid neoplasms, and are considered a distinct clinical entity. However, the pathogenesis of oversized parathyroid adenomas and the often-associated skeletal phenotype remains unclear. We present 5 cases of primary hyperparathyroidism (PHPT) with OFC, an uncommon manifestation of contemporary PHPT, associated with larger parathyroid adenomas, seen in the Bone and Mineral Disorders Clinic of the Henry Ford Health in the last 30 years to illustrate the critical role of vitamin D nutrition in the pathogenesis of both the OFC and adenoma size. The estimated prevalence of OFC was very low 0.2%, 5 of the >3000 surgically confirmed cases of PHPT seen during this time. The mean ± SD values were: age: 36.8 ± 22.1 years (4 of the 5 <36years), serum calcium 11.6 ± 1.1 mg/dl, alkaline phosphatase 799 ± 487 IU/L, PTH 1440 ± 477 pg/ml, 25-hydroxyvitamin D 13.0 ± 8.9 ng/ml, 1,25-dihyroxyvitamin D 26.5 ± 13.7 pg/ml, urine calcium 562 ± 274 mg/day, and parathyroid adenoma weight 4.53 ± 2.2 g. Parathyroidectomy led to the resolution of both the biochemical indices and OFC in each patient without recurrence over >10 years of follow-up. Because OFC is a very rare in the West, but very common areas of endemic vitamin D deficiency, we also examined the relationship between vitamin D nutrition, as assessed by serum 25-hydroxyvitamin D level, and parathyroid adenoma weight as well as prevalence of OFC in two large secularly diverse cohorts of patients with PHPT (Detroit, USA and Chandigarh, India). Based on this relationship and the relative prevalence of OFC in these two large cohorts, we propose that vitamin D nutrition (and perhaps calcium nutrition) best explains both the adenoma size and prevalence of OFC.
甲状旁腺腺瘤重量超过 3.5 克被报告为“非典型”、“大”或“巨大”甲状旁腺腺瘤。所有这些腺瘤都是罕见的变异，占所有甲状旁腺腺瘤的比例不超过 1.5%。大甲状旁腺腺瘤通常与更严重的疾病形式相关，包括纤维性骨炎囊性变（OFC），并具有良性和恶性甲状旁腺瘤的许多生化、组织学和分子特征，被认为是一种独特的临床实体。然而，超大甲状旁腺腺瘤的发病机制和常伴随的骨骼表型仍不清楚。我们报告了 5 例原发性甲状旁腺功能亢进症（PHPT）伴 OFC，这是当代 PHPT 的一种不常见表现，与更大的甲状旁腺腺瘤相关，这些病例均来自 Henry Ford Health 的骨骼和矿物质疾病诊所，在过去 30 年中观察到，以说明维生素 D 营养在 OFC 和腺瘤大小发病机制中的关键作用。OFC 的估计患病率非常低，为 0.2%，在这段时间内，超过 3000 例经手术证实的 PHPT 病例中只有 5 例。平均值±标准差为：年龄：36.8±22.1 岁（5 例中 4 例<36 岁），血清钙 11.6±1.1mg/dl，碱性磷酸酶 799±487IU/L，甲状旁腺素 1440±477pg/ml，25-羟维生素 D 13.0±8.9ng/ml，1,25-二羟维生素 D 26.5±13.7pg/ml，尿钙 562±274mg/天，甲状旁腺腺瘤重量 4.53±2.2g。甲状旁腺切除术使每位患者的生化指标和 OFC 都得到了缓解，且在 10 年以上的随访中无复发。由于 OFC 在西方非常罕见，但在维生素 D 缺乏的地方性流行地区非常常见，我们还研究了维生素 D 营养（通过血清 25-羟维生素 D 水平评估）与甲状旁腺腺瘤重量以及 PHPT 患者两个大型、具有时间差异的队列中 OFC 的患病率之间的关系（美国底特律和印度昌迪加尔）。基于这种关系以及这两个大队列中 OFC 的相对患病率，我们提出维生素 D 营养（或许还有钙营养）可以最好地解释腺瘤大小和 OFC 的患病率。
