Abstract

Context

Confirming a diagnosis of Cushing disease (CD) remains challenging, yet is critically important before recommending transsphenoidal surgery for adenoma resection.

Objective

To describe predictive performance of preoperative biochemical and imaging data relative to post-operative remission and clinical characteristics in patients with presumed CD.

Design, Setting, Patients, Interventions

Patients (n = 105; 86% female) who underwent surgery from 2007 through 2020 were classified into 3 groups: group A (n = 84) pathology-proven ACTH adenoma; group B (n = 6) pathology-unproven but with postoperative hypocortisolemia consistent with CD; and group C (n = 15) pathology-unproven, without postoperative hypocortisolemia. Group A + B were combined as confirmed CD and group C as unconfirmed CD.

Main outcomes

Group A + B was compared with group C regarding predictive performance of preoperative 24-hour urinary free cortisol (UFC), late night salivary cortisol (LNSC), 1-mg dexamethasone suppression test (DST), plasma ACTH, and pituitary magnetic resonance imaging (MRI).

Results

All groups had a similar clinical phenotype. Compared with group C, group A + B had higher mean UFC (P < 0.001), LNSC (P = 0.003), DST (P = 0.06), and ACTH (P = 0.03) and larger MRI-defined lesions (P < 0.001). The highest accuracy thresholds were: UFC 72 µg/24 hours; LNSC 0.122 µg/dL, DST 2.70 µg/dL, and ACTH 39.1 pg/mL. Early (3-month) biochemical remission was achieved in 76/105 (72%) patients: 76/90(84%) and 0/15(0%) of group A + B vs group C, respectively, P < 0.0001. In group A + B, nonremission was strongly associated with adenoma cavernous sinus invasion.

Conclusions

Use of strict biochemical thresholds may help avoid offering transsphenoidal surgery to presumed CD patients with equivocal data and improve surgical remission rates. Patients with Cushingoid phenotype but equivocal biochemical data warrant additional rigorous testing.

中文翻译：

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库欣病的诊断陷阱：105 名患者的手术缓解率、测试阈值和经验教训

摘要

语境

确认库欣病 (CD) 的诊断仍然具有挑战性，但在推荐经蝶手术切除腺瘤之前至关重要。

客观的

描述与假定 CD 患者术后缓解和临床特征相关的术前生化和影像学数据的预测性能。

设计、环境、患者、干预

从 2007 年到 2020 年接受手术的患者（n = 105；86% 女性）分为 3 组：A 组（n = 84）经病理学证实的 ACTH 腺瘤；B 组 (n = 6) 病理学未经证实，但术后低皮质醇血症与 CD 一致；和 C 组 (n = 15) 未经病理学证实，无术后低皮质醇血症。A + B 组合并为已确认的 CD，C 组为未确认的 CD。

主要成果

A + B 组与 C 组在术前 24 小时尿游离皮质醇 (UFC)、深夜唾液皮质醇 (LNSC)、1-mg 地塞米松抑制试验 (DST)、血浆促肾上腺皮质激素和垂体磁共振成像的预测性能方面进行比较（核磁共振）。

结果

所有组都有相似的临床表型。与 C 组相比，A + B 组的平均 UFC ( P  < 0.001)、LNSC ( P  = 0.003)、DST ( P  = 0.06) 和 ACTH ( P  = 0.03) 更高，MRI 定义的病灶更大 ( P  < 0.001 ）。最高准确度阈值为：UFC 72 µg/24 小时；LNSC 0.122 µg/dL、DST 2.70 µg/dL 和 ACTH 39.1 pg/mL。76/105 (72%) 名患者实现了早期（3 个月）生化缓解：A + B 组与 C 组分别为 76/90（84%）和 0/15（0%），P  < 0.0001。在 A + B 组中，未缓解与腺瘤海绵窦浸润密切相关。

结论

使用严格的生化阈值可能有助于避免为数据不明确的假定 CD 患者提供经蝶手术，并提高手术缓解率。具有库欣样表型但生化数据模棱两可的患者需要额外的严格测试。