Pacific Neuroscience Institute, John Wayne Cancer Institute at Providence's Saint John's Health Center, 2125 Arizona Ave., Santa Monica, CA, 90404, USA.
Pituitary. 2019 Aug;22(4):362-371. doi: 10.1007/s11102-019-00962-y.
Rathke's cleft cysts (RCCs) are common sellar lesions. Their management remains controversial, particularly when small or asymptomatic. Herein we review a consecutive series of RCC patients managed with surgery or observation.
All patients with a new diagnosis of presumed RCC, based on MRI, from February 2012-March 2018 were retrospectively divided into observational and surgical cohorts based on an intent-to-treat model. The cohorts were compared for clinical presentation, and cyst volume. The observational cohort was followed for change in cyst size. The surgical cohort was followed for changes in endocrinopathy, visual symptoms, headache and recurrence.
Of 90 patients (mean age 36.7 ± 19.4 years; 68% female), 60% (n = 54) were in the observational cohort and 40% (n = 36) in the surgical cohort. Average follow-up was 13 ± 23 months in the observational cohort and 24 ± 19 months in the surgical group. In comparing the cohorts, mean ages were similar with more women in the surgical group (81% vs. 56%, p = 0.04). Most patients in the observational cohort had incidentally-discovered RCCs (n = 50, 88%) as opposed to the surgical cohort (n = 6, 17%). The surgical cohort had higher rates of headache (89% vs 26%, p < 0.001), endocrinopathy (36% vs 0%, p < 0.001), and visual dysfunction (19% vs 0%, p = 0.001). Mean cyst volume and maximal cyst dimensions were greater in the surgical cohort (0.94 ± 0.77 cm and 14.2 ± 4.1 mm), compared to the observational cohort (0.1 ± 0.14 cm and 6.4 ± 3 mm), (p < 0.001). Among the 53% (n = 30/54) of patients in the observational group with follow-up, 3 (10%) had spontaneous RCC shrinkage, 1 (3%) had modest asymptomatic growth (at 10 months from initial MRI), and 87% had stable cyst size. Of the 36 patients recommended to have surgery, 89% (n = 32) did so. Post-operatively, complete or partial resolution of headache, endocrinopathy and visual dysfunction were documented in 90% (n = 28/30), 75% (n = 10/12), and 100% (n = 7/7), respectively. On follow-up MRI, 8 (22%) patients had some cyst reaccumulation, of whom 3 (8%) were symptomatic and underwent uneventful reoperation. No major complications such as hematoma, CSF leak, new endocrinopathy or visual deficits occurred.
From this consecutive series, a majority (60%) of RCCs do not appear to warrant surgical intervention and have a low risk of cyst progression. However, surgical cyst removal appears to be indicated and safe for patients with larger, symptomatic RCCs. Simple cyst drainage has a high rate of improvement in pituitary gland function, visual function and headache resolution with low complication rates and symptomatic recurrence risk. These findings stress the importance of careful case selection and potential utility of volumetric assessment for patients with RCCs.
Rathke 氏裂囊肿（RCC）是常见的鞍区病变。其治疗方法仍存在争议，尤其是对于体积较小或无症状的 RCC。在此，我们回顾了一系列接受手术或观察治疗的 RCC 患者。
根据 MRI 诊断为疑似 RCC 的所有患者（基于 MRI 诊断为疑似 RCC 的所有患者），根据意向治疗模型分为观察组和手术组。比较两组患者的临床表现和囊肿体积。观察组随访囊肿大小的变化。手术组随访内分泌变化、视力症状、头痛和复发情况。
90 例患者（平均年龄 36.7±19.4 岁；68%为女性）中，60%（n=54）入观察组，40%（n=36）入手术组。观察组平均随访时间为 13±23 个月，手术组为 24±19 个月。在比较两组时，平均年龄相似，但手术组女性患者比例更高（81%比 56%，p=0.04）。观察组（n=50，88%）多数患者为偶然发现的 RCC，而手术组（n=6，17%）则为有症状的 RCC。手术组头痛发生率较高（89%比 26%，p<0.001），内分泌紊乱发生率较高（36%比 0%，p<0.001），视觉功能障碍发生率较高（19%比 0%，p=0.001）。手术组囊肿体积和最大囊肿直径均大于观察组（0.94±0.77cm 和 14.2±4.1mm 比 0.1±0.14cm 和 6.4±3mm）（p<0.001）。在观察组 53%（n=30/54）有随访的患者中，3 例（10%）RCC 自发性缩小，1 例（3%）囊肿体积在 10 个月时出现轻微无症状性增大（MRI 初始时），87%患者囊肿体积稳定。在建议手术的 36 例患者中，89%（n=32）接受了手术。术后头痛、内分泌紊乱和视觉功能障碍完全或部分缓解的患者分别为 90%（n=28/30）、75%（n=10/12）和 100%（n=7/7）。随访 MRI 显示，8 例（22%）患者有一定程度的囊肿再积聚，其中 3 例（8%）有症状并接受了无并发症的再次手术。无主要并发症，如血肿、CSF 漏、新的内分泌或视力减退。
从这一系列连续病例中可以看出，大多数（60%）RCC 似乎不需要手术干预，且囊肿进展的风险较低。然而，对于体积较大、有症状的 RCC 患者，手术切除囊肿是有指征且安全的。简单的囊肿引流对于改善垂体功能、视觉功能和头痛缓解具有较高的成功率，且并发症发生率和有症状复发风险较低。这些发现强调了对 RCC 患者进行仔细病例选择的重要性，以及体积评估的潜在效用。
