Raue F, Kraimps J L, Dralle H, Cougard P, Proye C, Frilling A, Limbert E, Llenas L F, Niederle B
Medizinische Universitätsklinik, Heidelberg, Germany.
J Intern Med. 1995 Oct;238(4):369-73. doi: 10.1111/j.1365-2796.1995.tb01212.x.
The rarity of primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia type 2A (MEN 2A) led us to study clinical findings, surgical therapy and outcome in 67 patients in order to evaluate our therapeutic strategy. The retrospective study was based on cases registered by the EUROMEN study group (nine participating centres) from 1972 to 1993. Characteristics of PHPT in 67 patients (41 females, 26 males) with MEN 2A were reviewed. All patients underwent exploratory neck surgery; PHPT was confirmed histologically and/or biochemically. The median age at diagnosis of PHPT was 38 years. In 75% of the patients, PHPT and medullary thyroid carcinoma were diagnosed synchronously, while in 4%. PHT was diagnosed earlier. In 18% of the patients, PHPT was diagnosed after thyroidectomy, and in 3%, after discovery of pheochromocytoma. Primary hyperparathyroidism was asymptomatic in 84% of the patients; 15% suffered from renal stones. Serum calcium was slightly elevated in 69% (2.9 +/- 0.2 mmol-1) and normal in 16% of subjects. A single adenomectomy was performed in 42% of the patients, subtotal parathyroidectomy in 31% and total parathyroidectomy with autotransplantation in 16%. Independent from the extent of resection, cure was achieved in 94% of the patients, including 13% with hypoparathyroidism; hypercalcaemia persisted in 3% and no information was available in 3%. In an 8-year follow-up, hypercalcaemia recurred in 12% of the patients, although half had undergone parathyroidectomy totally or subtotally. CONCLUSION. MEN 2A-related PHPT is characterized by a mild hypercalcemia which is mostly asymptomatic and can be cured by simple resection of an enlarged parathyroid gland in most cases.
2A 型多发性内分泌腺瘤病（MEN 2A）中原发性甲状旁腺功能亢进症（PHPT）较为罕见，这促使我们对 67 例患者的临床症状、手术治疗及预后进行研究，以评估我们的治疗策略。这项回顾性研究基于欧洲 MEN 研究小组（9 个参与中心）1972 年至 1993 年登记的病例。我们回顾了 67 例（41 例女性，26 例男性）患有 MEN 2A 的 PHPT 患者的特征。所有患者均接受了颈部探查手术；PHPT 通过组织学和/或生化检查得以确诊。PHPT 确诊时的中位年龄为 38 岁。75%的患者中，PHPT 和甲状腺髓样癌同时被诊断出来，而 4%的患者中，PHPT 被更早诊断。18%的患者在甲状腺切除术后被诊断出 PHPT，3%的患者在发现嗜铬细胞瘤后被诊断出。84%的患者原发性甲状旁腺功能亢进症无症状；15%的患者患有肾结石。69%的受试者血清钙轻度升高（2.9±0.2 mmol/L），16%的受试者血清钙正常。42%的患者进行了单个腺瘤切除术，31%的患者进行了甲状旁腺次全切除术，16%的患者进行了甲状旁腺全切除术并自体移植。无论切除范围如何，94%的患者治愈，其中 13%患有甲状旁腺功能减退症；3%的患者高钙血症持续存在，3%的患者无相关信息。在 8 年的随访中，12%的患者高钙血症复发，尽管其中一半患者已接受了甲状旁腺全切除或次全切除术。结论：MEN 2A 相关的 PHPT 的特点是轻度高钙血症，大多无症状，多数情况下通过简单切除增大的甲状旁腺即可治愈。
