Craniopharyngioma medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Patients with craniopharyngioma have many treatment options. The selection depends on the size, location of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods.

Medical Therapy

For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.[1]

===Newly diagnosed craniopharyngioma===[2] There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma, in part because of the lack of prospective randomized trials that compare the different treatment options. Treatment is individualized on the basis of factors that include the following:

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

Treatment options for newly diagnosed craniopharyngioma include the following:

  • 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, even for sizeable tumors.
      • 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, accounting for some recurrences. Almost all craniopharyngiomas have an attachment to the pituitary stalk, and of the patients who undergo radical surgery, virtually all will require life-long pituitary hormone replacement with multiple medications.
  • Subtotal resection with radiation therapy
  • Primary cyst drainage with or without radiation therapy

Recurrent craniopharyngioma

  • Surgery
  • Radiation therapy, including radiosurgery
  • Intracavitary instillation of radioactive P-32, bleomycin, or interferon-alpha, for those with cystic recurrences
  • Systemic interferon

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