Craniopharyngioma surgery

Surgery is the mainstay of treatment for craniopharyngioma. Radical surgery is done either by transsphenoidal approach or by craniotomy. Recent studies propose subtotal resection with post operative radiotherapy as the management of choice for craniopharyngiomas, especially in the pediatric population. More advanced radiotherapy modalities currently under investigation include Gamma Knife and cyber knife radiosurgery.

• Surgery is the first-line treatment option for patients with craniopharyngioma, with or without radiation.
• For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.^[1]

• There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma.

• Treatment is individualized on the basis of factors that include the following: ^[2]

1. Tumor size
2. Tumor location
3. Extension of the tumor
4. Potential short-term and long-term toxicity

• Radical surgery with or without radiation therapy
  • It is possible to remove all visible tumor and achieve long-term disease control because these tumors are histologically benign.
  • A 5-year progression-free survival (PFS) rate of about 65% has been reported.
  • Radical surgical approaches include the following:
    • Transsphenoidal approach:
    • A transsphenoidal approach may be possible for some small tumors located entirely within the sella.
    • The development of expanded endonasal techniques with endoscopic visualization have allowed this approach to be increasingly used.
    • Craniotomy:
    • When an endonasal approach is not possible, a craniotomy is required.
    • The surgeon often has a limited view of the hypothalamic and sellar regions, and portions of the mass may remain after surgery.
    • Almost all craniopharyngiomas have an attachment to the pituitary stalk, and virtually all will require life-long pituitary hormone replacement.^[3]

• Subtotal resection with radiation therapy
  • The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. ^[3]
  • The surgical procedure is often followed by radiation therapy with 10-year overall survival rates higher than 90%.
  • Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention. ^{[2] [4]}
  • Surgical complications with subtotal resection are less likely than with radical surgery.

• Primary cyst drainage with or without radiation therapy
  • For larger cystic craniopharyngiomas, particularly in children younger than 3 years.
  • Benefits include temporary relief of fluid pressure by serial drainage, and in some cases, for intracystic instillation of sclerosing agents.
  • This procedure allows to use a two-staged approach: first draining the cyst via the implanted catheter, to relieve pressure and complicating symptoms and then later resecting the tumor or employing radiation therapy. ^[5]

• The incidence of tumor progression after subtotal surgical resection and radiotherapy ranges from 12-25%.
• Treatment options for recurrent childhood craniopharyngioma include the following: ^[6]

1. Surgery
2. Radiation therapy including radiosurgery

• The management of recurrent craniopharyngioma is determined largely by previous therapy.
• Repeat attempts at gross-total resections are difficult, and long-term disease control is less often achieved.
• Complications are more frequent than with initial surgery.^{[2] [6]}

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