Novodvorsky Peter, Hussein Ziad, Arshad Muhammad Fahad, Iqbal Ahmed, Fernando Malee, Munir Alia, Balasubramanian Sabapathy P
Department of Diabetes and Endocrinology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK.
Endocrinol Diabetes Metab Case Rep. 2019 May 7;2019. doi: 10.1530/EDM-18-0136.
Spontaneous remission of primary hyperparathyroidism (PHPT) due to necrosis and haemorrhage of parathyroid adenoma, the so-called 'parathyroid auto-infarction' is a very rare, but previously described phenomenon. Patients usually undergo parathyroidectomy or remain under close clinical and biochemical surveillance. We report two cases of parathyroid auto-infarction diagnosed in the same tertiary centre; one managed surgically and the other conservatively up to the present time. Case #1 was a 51-year old man with PHPT (adjusted (adj.) calcium: 3.11 mmol/L (reference range (RR): 2.20-2.60 mmol/L), parathyroid hormone (PTH) 26.9 pmol/L (RR: 1.6-6.9 pmol/L) and urine calcium excretion consistent with PHPT) referred for parathyroidectomy. Repeat biochemistry 4 weeks later at the surgical clinic showed normal adj. calcium (2.43 mmol/L) and reduced PTH. Serial ultrasound imaging demonstrated reduction in size of the parathyroid lesion from 33 to 17 mm. Twenty months later, following recurrence of hypercalcaemia, he underwent neck exploration and resection of an enlarged right inferior parathyroid gland. Histology revealed increased fibrosis and haemosiderin deposits in the parathyroid lesion in keeping with auto-infarction. Case #2 was a 54-year-old lady admitted with severe hypercalcaemia (adj. calcium: 4.58 mmol/L, PTH 51.6 pmol/L (RR: 1.6-6.9 pmol/L)) and severe vitamin D deficiency. She was treated with intravenous fluids and pamidronate and 8 days later developed symptomatic hypocalcaemia (1.88 mmol/L) with dramatic decrease of PTH (17.6 pmol/L). MRI of the neck showed a 44 mm large cystic parathyroid lesion. To date, (18 months later), she has remained normocalcaemic. Learning points: Primary hyperparathyroidism (PHPT) is characterised by excess parathyroid hormone (PTH) secretion arising mostly from one or more autonomously functioning parathyroid adenomas (up to 85%), diffuse parathyroid hyperplasia (<15%) and in 1-2% of cases from parathyroid carcinoma. PHPT and hypercalcaemia of malignancy, account for the majority of clinical presentations of hypercalcaemia. Spontaneous remission of PHPT due to necrosis, haemorrhage and infarction of parathyroid adenoma, the so-called 'parathyroid auto-infarction', 'auto-parathyroidectomy' or 'parathyroid apoplexy' is a very rare in clinical practice but has been previously reported in the literature. In most cases, patients with parathyroid auto-infarction undergo parathyroidectomy. Those who are managed conservatively need to remain under close clinical and biochemical surveillance long-term as in most cases PHPT recurs, sometimes several years after auto-infarction.
原发性甲状旁腺功能亢进症（PHPT）因甲状旁腺腺瘤坏死和出血而自发缓解，即所谓的“甲状旁腺自梗死”，是一种非常罕见但此前已有描述的现象。患者通常接受甲状旁腺切除术或接受密切的临床和生化监测。我们报告在同一三级医疗中心诊断出的两例甲状旁腺自梗死病例；一例接受了手术治疗，另一例至今采取保守治疗。病例1是一名51岁男性，患有PHPT（校正钙：3.11 mmol/L（参考范围（RR）：2.20 - 2.60 mmol/L），甲状旁腺激素（PTH）26.9 pmol/L（RR：1.6 - 6.9 pmol/L），尿钙排泄与PHPT一致），因该疾病接受甲状旁腺切除术。4周后在外科门诊复查生化指标显示校正钙正常（2.43 mmol/L）且PTH降低。系列超声成像显示甲状旁腺病变大小从33 mm减小至17 mm。20个月后，高钙血症复发，他接受了颈部探查并切除了肿大的右下甲状旁腺。组织学检查显示甲状旁腺病变中纤维化增加和含铁血黄素沉积增加，符合自梗死表现。病例2是一名54岁女性，因严重高钙血症（校正钙：4.58 mmol/L，PTH 51.6 pmol/L（RR：1.6 - 6.9 pmol/L））和严重维生素D缺乏入院。她接受了静脉补液和帕米膦酸治疗，8天后出现症状性低钙血症（1.88 mmol/L），PTH显著降低（17.6 pmol/L）。颈部MRI显示一个44 mm大的囊性甲状旁腺病变。迄今为止（18个月后），她的血钙一直保持正常。经验教训：原发性甲状旁腺功能亢进症（PHPT）的特征是甲状旁腺激素（PTH）分泌过多，主要源于一个或多个自主功能的甲状旁腺腺瘤（高达85%）、弥漫性甲状旁腺增生（<15%），在1 - 2%的病例中源于甲状旁腺癌。PHPT和恶性肿瘤性高钙血症占高钙血症临床病例的大多数。因甲状旁腺腺瘤坏死、出血和梗死导致的PHPT自发缓解，即所谓的“甲状旁腺自梗死”、“自体甲状旁腺切除术”或“甲状旁腺卒中”，在临床实践中非常罕见，但此前文献已有报道。在大多数情况下，甲状旁腺自梗死患者接受甲状旁腺切除术。那些接受保守治疗的患者需要长期接受密切的临床和生化监测，因为在大多数情况下PHPT会复发，有时在自梗死数年之后。
