From the Guidelines
The primary treatment for adamantinomatous craniopharyngioma is surgical resection, with the goal of complete tumor removal when possible, followed by proton beam therapy as a weakly recommended postoperative radiotherapy for unresectable or postoperative persistent tumors, as suggested by the JASTRO and JSPHO guidelines 1.
Treatment Approach
The treatment of adamantinomatous craniopharyngioma involves a multimodal approach, including:
- Surgical resection: The goal is to achieve complete tumor removal when possible, using approaches such as transsphenoidal, transcranial, or endoscopic techniques, depending on tumor size and location.
- Radiation therapy: Often recommended after surgery, particularly for subtotal resections, to target residual tumor cells and reduce recurrence risk.
- Proton beam therapy (PBT): Weakly recommended as postoperative radiotherapy for unresectable or postoperative persistent craniopharyngioma, with local control, progression-free survival, and overall survival rates similar to those of conventional X-ray therapy 1.
- Intracystic treatments: For cystic components, treatments such as instillation of bleomycin, interferon alpha, or radioactive phosphorus (32P) may be employed to shrink the cyst.
- Hormone replacement therapy: Frequently necessary postoperatively, as these tumors often affect the hypothalamic-pituitary axis, requiring lifelong supplementation of hormones like cortisol, thyroid hormone, growth hormone, and sex hormones.
- Long-term follow-up: Essential to monitor for recurrence, which can occur even years after initial treatment, using serial MRI imaging.
Rationale
The use of proton beam therapy as a postoperative radiotherapy is supported by the JASTRO and JSPHO guidelines, which conclude that PBT can provide a therapeutic effect equal to that of conventional X-ray therapy, with a level of evidence evaluated as C 1. While there is no randomized comparative study of X-ray therapy and PBT, several reports have made this comparison, and it was concluded that PBT has the potential to decrease adverse events with further long-term studies 1. Therefore, proton beam therapy is a valid treatment option for craniopharyngioma, particularly for unresectable or postoperative persistent tumors.
From the Research
Treatment Options for Adamantinomatous Craniopharyngioma
The treatment for adamantinomatous craniopharyngioma (ACP) typically involves a combination of surgical resection, radiation therapy, and targeted therapies. The goal of treatment is to remove the tumor while minimizing damage to surrounding brain tissue and preserving quality of life.
Surgical Resection
- Surgical resection is the primary treatment for ACP, with the goal of removing as much of the tumor as possible while preserving surrounding brain tissue 2, 3.
- Radical surgery can provide good tumor control, but it also carries a high risk of permanent morbidity, including endocrine, cognitive, and neurological deficits 3.
Radiation Therapy
- Radiation therapy may be used as an adjuvant treatment to reduce the risk of tumor recurrence after surgical resection 3.
- Proton therapy has been shown to be effective in treating ACP, with reduced risk of damage to surrounding brain tissue 3.
Targeted Therapies
- Targeted therapies, such as bevacizumab and tocilizumab, have been shown to be effective in stabilizing or shrinking ACP tumors, and may be used in combination with surgery and radiation therapy 2, 4, 5.
- IL-6 and vascular endothelial growth factor (VEGF) have been identified as potential therapeutic targets for ACP, and agents that target these pathways are being investigated in clinical trials 4, 5.