Abstract
Craniopharyngiomas (CPs) are benign tumors arising from the sellar region. However, little is known about their clinical features and long-term recurrence due to low morbidity and the lack of large cohort studies. Thus, we aimed to develop nomograms to accurately predict the extent of resection and tumor recurrence using clinical parameters. A total of 545 patients diagnosed with CP between 2009 and 2019 were examined: 381 in the development cohort and 164 in the validation cohort. Least absolute shrinkage and selection operator (LASSO) and Cox regression analyses were performed to establish two nomograms. Receiver operating characteristic (ROC) curves, calibration curves, decision curve analysis (DCA) and Kaplan-Meier (KM) curves were used to evaluate their predictive performance and discriminative power, respectively, in the two cohorts. In addition, the EORTC QLQ-BN20 questionnaire was used to assess neuropsychological status in the follow-up. In the development cohort, the area under the curve (AUC) and C-index were 0.760 and 0.758, respectively, for predicting the extent of resection and 0.78 and 0.75, respectively, for predicting 3-year progression-free survival (PFS) and 5-year PFS. Additionally, the model had a predictive accuracy of 0.785. Both nomograms showed acceptable discrimination in the two cohorts. Moreover, DCA demonstrated excellent clinical benefits from the two nomograms. Finally, participants were classified into two distinct risk groups according to the risk score, and an online calculator was created for convenient clinical use. During long term follow-up, hypothyroidism (77.61%) and hypocortisolism (76.70%) were the most common endocrine dysfunction after surgery and significant deficits were observed concerning visual disorder, motor dysfunction and seizures in the recurrent groups. In particular, better quality of life was associated with gross total resection (GTR), postoperative radiation, anterior interhemispheric (AI) approach and transsphenoidal approach. To our knowledge, these are the first nomograms based on a very large cohort of patients with CP that show potential benefits for guiding treatment decisions and long-term surveillance. The current study demonstrated the online calculator serve as the practical tool for individual strategies based on the patient’s baseline characteristics to achieve a better prognosis.
Highlights
Craniopharyngiomas (CPs) are benign suprasellar tumors accounting for 2-4% of intracranial tumors [1, 2]
A total of 545 patients were included in this group of studies: 381 in the training cohort and 164 in the validation cohort
We further explored the impact of different approaches on terminal BN20 scores
Summary
Craniopharyngiomas (CPs) are benign suprasellar tumors accounting for 2-4% of intracranial tumors [1, 2]. Histologically classified as WHO I tumors, total resection and postoperative management are huge challenges associated with CPs because they are adjacent to vital brain structures, such as the optic chiasm and hypothalamus. Radical resection is considered the first-line treatment because it yields the best overall survival (OS) and progression-free survival (PFS). Complete resection could lead to increased mortality or poor functional results because of severe endocrine disorders [3]. The rate of long-term recurrence could reach an astonishing 58% with or without radiotherapy in the subtotal resection group of some studies [4], so subsequent treatment often becomes an unavoidable problem for such patients. Salvage radiotherapy or intratumoral chemotherapy [5, 6] can be carried out in relapsed patients, though its therapeutic effect is not satisfactory
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