Department of Neurosurgery, LMU University Hospital, LMU Munich, Marchioninistr 15, 81377, Munich, Germany.
Acta Neurochir (Wien). 2023 Sep;165(9):2435-2444. doi: 10.1007/s00701-023-05733-0. Epub 2023 Aug 2.
Although Rathke cleft cysts (RCC) are benign lesions of the sellar region, recurrence is frequent after surgical treatment. Nuclear translocation of ß-catenin (NTßC), a key effector of the wnt-signaling pathway that is responsible for cell renewal, has been shown to act as a proto-oncogene and is considered to be a potential risk factor for increased recurrence in RCC. In this study, we analyzed a surgically treated cohort into patients with and without NTßC expression in order to identify clinical and imaging differences and further evaluate the risk of recurrence.
Patients with resection of RCC between 04/2001 and 11/2020 were included. Histological specimens were immunohistochemically stained for ß-catenin. Study endpoints were time to cyst recurrence (TTR) and functional outcome. Functional outcome included ophthalmological and endocrinological data. Furthermore, MRI data were assessed.
Seventy-three patients (median age 42.3 years) with RCC underwent mainly transsphenoidal cyst resection (95.9%), 4.1% via transcranial approach. Immunohistochemical staining for ß-catenin was feasible in 61/73 (83.6%) patients, with nuclear translocation detected in 13/61 cases (21.3%). Patients with and without NTßC were equally likely to present with endocrine dysfunction before surgery (p = 0.49). Postoperative new hypopituitarism occurred in 14/73 (19.2%) patients. Preoperative visual impairment was equal in both groups (p = 0.52). Vision improved in 8/21 (33.3%) patients and visual field deficits in 22/34 (64.7%) after surgery. There was no difference in visual and perimetric outcome between patients with and without NTßC (p = 0.45 and p = 0.23, respectively). On preoperative MRI, cyst volume (9.9 vs. 8.2 cm; p = 0.4) and evidence of hemorrhage (30.8% vs. 35.4%; p = 0.99) were equal and postoperative cyst volume decreased significantly in both groups (0.7 vs. 0.5 cm; p < 0.0001 each). Cyst progression occurred in 13/73 (17.8%) patients after 39.3 ± 60.3 months. Cyst drainage with partial removal of the cyst wall resulted in improved recurrence-free survival without increasing the risk of complications compared with cyst fenestration alone. Patients with postoperative diabetes insipidus had an increased risk for recurrence according to multivariate analysis (p = 0.005). NTßC was evident in 4/15 patients (26.7%) and was not associated with a higher risk for recurrence (p = 0.67).
Transnasal transsphenoidal cyst drainage with partial removal of the cyst wall reduces the risk of recurrence without increasing the risk of complications compared with fenestration of the cyst alone. Patients with postoperative diabetes insipidus seem to have an increased risk for recurrence. In contrast, NTßC was not associated with a higher risk of recurrence and did not provide stratification for clinically distinct patients.
尽管 Rathke 裂隙囊肿（RCC）是鞍区的良性病变，但手术后复发率很高。β-连环蛋白（β-catenin）的核易位（NTβC）是 Wnt 信号通路的关键效应物，负责细胞更新，已被证明作为原癌基因发挥作用，被认为是 RCC 复发风险增加的潜在危险因素。在本研究中，我们分析了一组接受手术治疗的患者，以确定是否存在 NTβC 表达，以识别临床和影像学差异，并进一步评估复发风险。
纳入 2001 年 4 月至 2020 年 11 月期间接受 RCC 切除术的患者。组织学标本通过免疫组织化学染色β-catenin。研究终点为囊肿复发时间（TTR）和功能结局。功能结局包括眼科和内分泌学数据。此外，还评估了 MRI 数据。
73 例 RCC 患者（中位年龄 42.3 岁）主要行经蝶窦囊肿切除术（95.9%），4.1%经颅入路。61/73（83.6%）例患者的β-catenin 免疫组织化学染色可行，13/61 例（21.3%）检测到核易位。有和无 NTβC 的患者术前发生内分泌功能障碍的可能性相同（p=0.49）。73 例患者中有 14 例（19.2%）术后新发垂体功能减退。两组术前视力障碍相同（p=0.52）。21 例患者中有 8 例（33.3%）视力改善，34 例患者中有 22 例（64.7%）视野缺损改善。术后两组患者视力和视野结果无差异（p=0.45 和 p=0.23）。术前 MRI 显示囊肿体积（9.9 vs. 8.2cm；p=0.4）和出血证据（30.8% vs. 35.4%；p=0.99）相同，两组术后囊肿体积均明显减少（0.7 vs. 0.5cm；p<0.0001 各）。73 例患者中有 13 例（17.8%）在 39.3±60.3 个月后囊肿进展。与单纯囊肿开窗术相比，囊肿引流联合部分囊壁切除可降低复发风险，而不增加并发症风险。多变量分析显示，术后发生尿崩症的患者复发风险增加（p=0.005）。15 例患者中有 4 例（26.7%）存在 NTβC，与较高的复发风险无关（p=0.67）。
与单纯囊肿开窗术相比，经鼻蝶窦囊肿引流联合部分囊壁切除可降低复发风险，而不增加并发症风险。术后发生尿崩症的患者复发风险似乎增加。相反，NTβC 与较高的复发风险无关，并且不能对临床特征明显不同的患者进行分层。
