2Mayo Clinic Alix School of Medicine, Rochester, Minnesota.
Departments of1Neurologic Surgery.
Neurosurg Focus. 2020 Jun;48(6):E3. doi: 10.3171/2020.3.FOCUS2084.
Cushing's disease arises from functioning adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing's disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing's disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing's disease.
A single-institution database of Cushing's disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing's disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI.
Of the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61-6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002).
Empty sella syndrome is associated with an increased rate of MRI-negative Cushing's disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.
库欣病是由功能性促肾上腺皮质激素（ACTH）分泌垂体腺瘤引起的。这些肿瘤可能非常小，并且可以逃避 MRI 的检测。空泡蝶鞍综合征是一种蛛网膜脑脊液向外膨出进入蝶鞍导致垂体受压的现象，可能是由于颅内压升高（库欣病的常见问题），进一步导致垂体难以可视化，这可能导致在 MRI 上发现肿瘤变得困难，即所谓的 MRI 阴性库欣病。作者试图研究空泡蝶鞍综合征与 MRI 阴性库欣病之间的关系。
回顾了 2000 年至 2017 年的单一机构库欣病病例数据库，共纳入 197 例病例进行分析。180 例患者有库欣病的组织学诊断，17 例患者通过手术缓解，但未获得明确的组织学诊断。排除大腺瘤（肿瘤 > 1 厘米）。空泡蝶鞍综合征的程度根据中线矢状 T1 加权 MRI 中蝶鞍内脑脊液的可见程度进行分级。
在所确定的 197 例病例中，有 40 例（20%）表现为 MRI 阴性疾病，有 49 例（25%）存在空泡蝶鞍综合征。在 49 例空泡蝶鞍病例中，MRI 阴性疾病发现 18 例（37%），而在 148 例无空泡蝶鞍综合征的病例中发现 22 例（15%）。空泡蝶鞍综合征与 MRI 阴性疾病显著相关（OR 3.32，95%CI 1.61-6.74，p = 0.0018）。垂体厚度减少也与 MRI 阴性疾病相关（平均厚度 5.6 毫米与 6.8 毫米，p = 0.0002）。
空泡蝶鞍综合征与 MRI 阴性库欣病的发生率增加有关。垂体受压导致垂体体积相对减少，这可能是 MRI 未能检测到肿瘤的原因。
