Long-term outcome of surgically treated and conservatively managed Rathke cleft cysts.

OBJECTIVE: Rathke cleft cysts (RCC) are benign lesions of the sellar region that require surgical treatment in case of visual deterioration or progression of the cyst. However, the natural course is often stable and asymptomatic. We aimed to investigate the characteristics of patients with cyst progression during follow-up (FU) and to compare the natural history of patients with RCC with patients who underwent surgery. METHODS: Patients with an MR morphologic cystic sellar lesion classified as RCC between 04/2001 and 11/2020 were included. Functional outcomes, including ophthalmologic, endocrinologic, and MRI data, were retrospectively analyzed and compared between surgically treated patients, patients on a "watch and wait" strategy (WWS), and patients on a WWS who underwent secondary surgery due to cyst progression. RESULTS: One hundred forty patients (median age 42.8 years) with RCC on MRI were identified. 52/140 (37.1%) underwent primary surgery. Of 88 patients (62.9%) with initial WWS, 21 (23.9%) underwent surgery for secondary cyst progression. Patients on the WWS had significantly smaller cyst volumes (p = 0.0001) and fewer visual disturbances (p = 0.0004), but a similar rate of hormone deficiencies (p = 0.99) compared with surgically treated patients preoperatively. Postoperatively patients suffered significantly more often from hormone deficiencies than WWS patients (p = 0.001). Patients who switched to the surgical group were significantly more likely to have preoperative T1 hyperintense signals on MRI (p = 0.0001) and visual disturbances (p = 0.001) than patients with continuous WWS. Postoperatively, these patients suffered more frequently from new hormonal deficiencies (p = 0.001). Endocrine and ophthalmologic outcomes in patients with primary and secondary surgery were comparable. Multivariate analysis showed that WWS patients were at a higher risk of requiring surgery for cyst progression when perimetric deficits (p = 0.006), hyperprolactinemia (p = 0.003), and corticotropic deficits (p = 0.005) were present. CONCLUSION: Surgical treatment of RCC may cause new hormonal deficiencies, which are rare in the natural course. Therefore, the indication for surgery should be carefully evaluated. Hyperprolactinemia and corticotropic deficits were significant indicators for a secondary cyst progression in patients with RCC. However, a significant amount of almost 25% of initially conservatively managed cysts showed deterioration, necessary for surgical intervention.

Surgery for prolactinomas: a better choice?

PURPOSE: Renewed interest in transsphenoidal surgery (TSS) as a therapeutic option for prolactinomas has emerged. METHODS: Based on contemporary literature and own experience, the changing role of surgery for treatment of prolactinomas is discussed. RESULTS: Today, TSS is performed by minimally invasive microscopic or endoscopic techniques. Normoprolactinemia is obtained in 71-100% of patients with microprolactinomas by TSS. Almost equal results are found in circumscribed intrasellar macroprolactinomas. In experienced hands, pituitary function is preserved in TSS. The risk of cardiac valve disease is still a concern with ergot-derived dopamine-agonists (DAs) in patients requiring long-term, high-dose dopamine-agonist (DA) treatment. Cost-utility analysis favors TSS over DA treatment. The possible negative impact of DA treatment on future surgical results is still a controversial and unsettled issue. In patients who wish to become pregnant, the advantages of microprolactinoma removal to avoid DAs and macroprolactinoma debulking to avoid symptomatic enlargement during pregnancy should be discussed with the patients. Young patients' age is an argument for surgery to circumvent the unpredictable sequelae of long-term DA treatment. Surgery should be discussed in male gender because of a higher likelihood of DA resistance and aggressive behavior of prolactinoma. CONCLUSION: Given excellent results of TSS and concerns about medical treatment, the scale of indications for TSS as an alternative to DAs has increased. The patient's wishes concerning a chance at a cure with TSS instead of a long-term treatment with DAs has become an important and accepted indication. With DA medication and TSS, two effective treatment modalities for prolactinomas are available that can be used in a complementary fashion.

Risk-benefit analysis of surgical treatment strategies for cystic craniopharyngioma in children and adolescents.

Objective: Treatment strategies for craniopharyngiomas are still under debate particularly for the young population. We here present tumor control and functional outcome data after surgical treatment focusing on stereotactic and microsurgical procedures for cystic craniopharyngiomas in children and adolescents. Methods: From our prospective institutional database, we identified all consecutive patients less than 18 years of age who were surgically treated for newly-diagnosed cystic craniopharyngioma between, 2000 and, 2022. Treatment decisions in favor of stereotactic treatment (STX) or microsurgery were made interdisciplinary. STX included aspiration and/or implantation of an internal shunt catheter for permanent cyst drainage. Microsurgery aimed for safe maximal tumor resections. Study endpoints were time to tumor recurrence (TTR) and functional outcome including ophthalmological/perimetric, endocrinological, and body-mass index (BMI) data. Results: 29 patients (median age 9.9 yrs, range 4-18 years) were analyzed. According to our interdisciplinary tumor board recommendation, 9 patients underwent stereotactic treatment, 10 patients microsurgical resection, and 10 patients the combination of both. Significant volume reduction was particularly achieved in the stereotactic (p=0.0019) and combined subgroups (p<0.001). Improvement of preoperative visual deficits was always achieved independent of the applied treatment modality. Microsurgery and the combinational treatment were associated with higher rates of postoperative endocrinological dysfunction (p<0.0001) including hypothalamic obesity (median BMI increase from 17.9kg/m2 to 24.1kg/m2, p=0.019). Median follow-up for all patients was 93.9 months (range 3.2-321.5 months). Recurrent tumors were seen in 48.3% and particularly concerned patients after initial combination of surgery and STX (p=0.004). In here, TTR was 35.1 ± 46.9 months. Additional radiation therapy was found indicated in 4 patients to achieve long-lasting tumor control. Conclusion: In children and adolescents suffering from predominantly cystic craniopharyngiomas, stereotactic and microsurgical procedures can improve clinical symptoms at low procedural risk. Microsurgery, however, bears a higher risk of postoperative endocrine dysfunction. A risk-adapted surgical treatment concept may have to be applied repeatedly in order to achieve long-term tumor control even without additional irradiation.

Factors predicting outcomes of endoscopic endonasal approach in craniopharyngioma patients.

Objectives: Endoscopic endonasal approach (EEA) is commonly used for resection of craniopharyngioma (CP). Treatment outcomes of EEA for CP were related to numerous factors; however, they have been evaluated in few studies. The objective of this study is to investigate factors associated with the outcomes of CP following this operation. Materials and Methods: The records of patients with CP, who underwent EEA at our institution from January 2014 to June 2022, were retrospectively reviewed. Surgical outcomes, including the extent of resection, visual recovery, and endocrinological outcomes, were reported. Clinical and radiographic factors were analyzed for their associations with treatment outcomes using logistic regression analyzes. Results: This study cohort consisted of 28 patients with CP. Gross total resection (GTR) was achieved in 12 patients (43%). Post-operative visual status improved, stabilized, and deteriorated in 89%, 6%, and 6% of the patients, respectively. There were no patients recovered from pre-operative pituitary dysfunctions, while post-operative hypoadrenalism, hypothyroidism, and hypogonadism were found in 9 (36%), 11 (42%), and 4 (22%) patients, respectively. Post-operative permanent diabetic insipidus was found in 13 patients (50%). Greater suprasellar extension of the tumor was associated with a lower rate of GTR (P = 0.011). Diabetes mellitus (DM) was associated with poor visual recovery (P = 0.022). Larger tumor size and Puget grade 2 were associated with postoperative hypoadrenalism (P = 0.01 and 0.023, respectively). In addition, Puget grade 2 was associated with post-operative hypothyroidism (P = 0.017). Conclusion: For EEA in CP, the extent of resection could be determined by suprasellar extension of the tumor. DM was a poor predicting factor for visual recovery, while larger tumors and Puget grade 2 had a higher risk of post-operative hypopituitarism.