Pineal tumours represent a valuable model of multidisciplinary collaboration. Over recent years, such collaboration has led to the significant improvements in outcomes, increased cure rates and reduction in long-term sequelae. Advances in diagnostic workup, including detailed tumour investigation and the study of tumoural markers, now allow more accurate treatment strategies. When a biopsy is required, a clear increase in the use of the endoscopic techniques has been observed in recent years, especially in paediatric patients. This approach allows simultaneous treatment of intracranial hypertension and establishment of a histological diagnosis. In cases of benign tumours, complete surgical removal should be the goal to ensure definitive cure. Advances in surgical techniques, including microsurgery, endoscopy-assisted surgery and the use of exoscopes, as well as progress in neuro-anaesthesiology have reinforced the concept that aggressive surgical resection represents a cornerstone of pineal tumour treatment when surgery is indicated. Results are less favourable for pinealoblastomas in both children and adults, but biomolecular and genetic studies have enabled the identification of different prognostic groups, allowing treatment intensification or de-escalation, thereby improving results while reducing the risk of treatment-related complications. The choice of complementary treatment based on histological diagnosis, biomolecular and genetic tumour markers and the possibility of tailored therapies may further improve survival and clinical outcomes in the future. Gamma Knife radiosurgery may be considered as an adjuvant or alternative treatment when surgery is contraindicated. However, the relatively high incidence of complications related to radionecrosis suggests that Gamma Knife should be used with caution, particularly in paediatric patients. Finally, to better understand the natural history of these rare tumours and the outcomes of surgical and complementary treatments, including chemotherapy and/or radiotherapy, it would be valuable to establish international registries. Although costly, such registries would provide substantial benefits for patients and their families.

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Conclusions

  • Carmine Mottolese

摘要

Pineal tumours represent a valuable model of multidisciplinary collaboration. Over recent years, such collaboration has led to the significant improvements in outcomes, increased cure rates and reduction in long-term sequelae. Advances in diagnostic workup, including detailed tumour investigation and the study of tumoural markers, now allow more accurate treatment strategies. When a biopsy is required, a clear increase in the use of the endoscopic techniques has been observed in recent years, especially in paediatric patients. This approach allows simultaneous treatment of intracranial hypertension and establishment of a histological diagnosis. In cases of benign tumours, complete surgical removal should be the goal to ensure definitive cure. Advances in surgical techniques, including microsurgery, endoscopy-assisted surgery and the use of exoscopes, as well as progress in neuro-anaesthesiology have reinforced the concept that aggressive surgical resection represents a cornerstone of pineal tumour treatment when surgery is indicated. Results are less favourable for pinealoblastomas in both children and adults, but biomolecular and genetic studies have enabled the identification of different prognostic groups, allowing treatment intensification or de-escalation, thereby improving results while reducing the risk of treatment-related complications. The choice of complementary treatment based on histological diagnosis, biomolecular and genetic tumour markers and the possibility of tailored therapies may further improve survival and clinical outcomes in the future. Gamma Knife radiosurgery may be considered as an adjuvant or alternative treatment when surgery is contraindicated. However, the relatively high incidence of complications related to radionecrosis suggests that Gamma Knife should be used with caution, particularly in paediatric patients. Finally, to better understand the natural history of these rare tumours and the outcomes of surgical and complementary treatments, including chemotherapy and/or radiotherapy, it would be valuable to establish international registries. Although costly, such registries would provide substantial benefits for patients and their families.