Radical Surgery
Limited Surgery and Radiation Therapy
Intracavitary Radiation Therapy and/or Chemotherapy
Stereotactic Radiation Therapy

There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma. No scientifically valid study has been performed to compare the different modalities in terms of recurrence rate or quality of life.[1] For this reason, treatment is determined for each patient individually.

Because these tumors are histologically benign, it may be possible to remove all the visible tumor and achieve a cure. Many surgical approaches have been described, and the route should be determined by the size, location, and extension of the tumor. A transsphenoidal approach may be possible in some small tumors located entirely within the sella, but this is not usually possible in children. The most common approach is craniotomy.

Radical surgery is technically challenging because the tumor is surrounded by vital structures, including the optic nerves and chiasm, the carotid artery and its branches, the hypothalamus, and the third cranial nerve. The tumor may be adherent to these structures, which may cause complications, and may limit the ability to remove all the tumor. The surgeon often has limited visibility in the region of the hypothalamus and in the sella, and portions of the mass may be left in these areas, 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.[2]

Complications of radical surgery include death from intraoperative hemorrhage, hypothalamic damage, or stroke. In addition to the need for hormone replacement, other complications include obesity, alteration in mood, blindness, seizures, spinal fluid leak, false aneurysms, [3] and difficulty with eye movements. Repeat surgery is considered more risky than the initial procedure.

If the surgeon feels that tumor remains, or if postoperative imaging reveals residual craniopharyngioma that is not resectable, radiation therapy will be required to prevent early progression. Periodic surveillance magnetic resonance imaging is performed for several years after radical surgery because of the possibility of tumor recurrence.

The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. No attempt is made to remove tumor from the pituitary stalk or hypothalamus. The surgical procedure is followed by radiation therapy. Conventional radiation is fractionated external-beam radiation with a recommended dose of 54 Gy to 55 Gy in 1.8 Gy fractions.[4] Surgical complications are less likely than with radical surgery. Complications of radiation include loss of pituitary hormonal function, development of late strokes and vascular malformations, delayed blindness, and development of second tumors within the radiation field. Tumor progression remains a possibility, and it is usually not possible to repeat the radiation dose.

Some craniopharyngiomas with a large cystic component may be treated by stereotaxic delivery of 32P, a radioactive substance with a very short penetration. This is usually considered for recurrent tumors.[5]

A single large dose of radiation is delivered to a very small field. Proximity of the craniopharyngioma to vital structures limits this to very small tumors that are in the sella.[5]

References

1. Sanford RA: Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg 21 (Suppl 1): 39-43, 1994.  [PUBMED Abstract]
   
   
2. Sands SA, Milner JS, Goldberg J, et al.: Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma. J Neurosurg 103 (4 Suppl): 302-11, 2005.  [PUBMED Abstract]
   
   
3. Sutton LN: Vascular complications of surgery for craniopharyngioma and hypothalamic glioma. Pediatr Neurosurg 21 (Suppl 1): 124-8, 1994.  [PUBMED Abstract]
   
   
4. Wara WM, Sneed PK, Larson DA: The role of radiation therapy in the treatment of craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 98-100, 1994.  [PUBMED Abstract]
   
   
5. Lunsford LD, Pollock BE, Kondziolka DS, et al.: Stereotactic options in the management of craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 90-7, 1994.  [PUBMED Abstract]
   
   

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