Surgery for Craniopharyngiomas Treatment & Management
- Author: Lawrence S Chin, MD, FACS; Chief Editor: Brian H Kopell, MD more...
No primary medical therapy exists for this tumor. Hormonal replacements are administered as needed if endocrine abnormalities exist.
The initial surgical decision concerns the approach. If the predominant portion of the tumor is intrasellar, the approach is usually transsphenoidal. Often, the suprasellar component can be delivered into the sella and evacuated. Further, the transsphenoidal approach is well tolerated by patients and is preferable to a craniotomy, when feasible.[19, 20, 21, 22, 23, 24, 25, 26]
If the pituitary sella is not enlarged, the transsphenoidal approach is generally not preferred. A craniotomy is usually necessary when the predominant component is suprasellar, although certain suprasellar masses may be located through an extended transsphenoidal approach. New technology in optics and instrumentation has now permitted many transsphenoidal procedures to be done exclusively through the endoscope. Specialized training is needed in these techniques, but in some instances, even large tumors in the suprasellar space can be safely removed or debulked.[27, 28, 29]
The pterional craniotomy is the standard craniotomy approach to suprasellar lesions because it allows good visualization of the optic nerves, chiasm, and surrounding structures. Variations of the pterional craniotomy have been proposed to include resection of the orbital rim and zygoma so as to provide a more defined view of the skull base and thereby allow better access to the superior aspects of this tumor. These approaches lend themselves to less frontal lobe retraction in order to visualize the operative site.
A subfrontal approach is appropriate for lesions that lie anterior to the optic chiasm, but this may be difficult to determine preoperatively. Under rare circumstances, a transcallosal approach is necessary when the tumor is entirely within the third ventricle. The drawback to this approach is the inability to identify the optic chiasm and pituitary stalk early in the dissection.
Cystic tumors are amenable to either a transsphenoidal approach or a pterional craniotomy. The solid components often adhere to the optic chiasm or hypothalamus and, therefore, may be difficult to remove in their entirety.
Cyst aspiration combined with intracavitary phosphorus-32 (32 P) instillation is an alternative to traditional surgical resection (see video below). Good long-term control of tumor growth has been demonstrated; however, a tumor with significant solid components is not likely to respond to32 P. Intracystic chemotherapy with bleomycin has also been tried, with some success in short-term reduction of cyst size. Intracavitary therapy requires that an Ommaya reservoir be placed into the cyst. Care needs to be taken that the catheter tip openings are in the cyst itself and that there is no spillage of cyst contents or injected material outside the cyst wall.
In an attempt to balance the advantages of an aggressive surgical resection against the risk of significant morbidity, a preoperative grading system (for children) has been proposed. This system considers the extent of invasion of the hypothalamus by the tumor and is as follows:
Type 0: The tumor represents no hypothalamic involvement.
Type 1: The tumor distorts or elevates the hypothalamus, but the latter is still visible.
Type 2: The hypothalamus is no longer visible.
Consequently, some proposed that a gross total resection be attempted in type 0 and type 1 tumors and that a subtotal resection be attempted in a type 2 tumor, leaving only the hypothalamic component. Although this grading system was developed in a pediatric population, applying it to an adult population is certainly feasible. Some evidence exists, however, that the craniopharyngiomas that arise in adults are less likely to invade the hypothalamus.
A newer classification system proposed by Kassam et al uses a scheme that divides tumors according to their suprasellar extension, based upon the endoscopic expanded endonasal approach (EEA), and is as follows:
Type I - Preinfundibular
Type II - Transinfundibular (extending into the stalk)
Type III - Retroinfundibular, extending behind the gland and stalk, and has 2 subdivisions (IIIa, extending into the third ventricle; and IIIb, extending into the interpeduncular cistern)
Type IV - Isolated to the third ventricle and/or optic recess and is not accessible via an endonasal approach
Radiation is indicated in patients who had a subtotal resection of craniopharyngioma or with recurrence of the disease. Types of radiation therapy include stereotactic radiosurgery (SRS) and fractionated external beam radiation therapy.
Stereotactic techniques (SRS) are based on localization of the target through stereotactic frame coordinates (attached to the head as in Gamma Knife) or non–frame-based techniques (CyberKnife). Small tumors can be treated with a single fraction of radiation that delivers a high dose of radiation with rapid falloff to minimize the dose to surrounding structures. A larger target volume or proximity to the optic nerve and chiasm may require use of fractionated SRS techniques designed to minimize damage to healthy structures. The dose delivered is usually limited by the optic chiasm, which ideally should receive less than 10 Gy.
External beam radiation therapy uses 3-dimensional planning to deliver radiation with a margin around the tumor. Up to 30 fractions may be used to deliver an effective total dose while keeping daily doses low. Recurrence of tumor is higher when total dose is less than 54 Gy, while complications increase with doses greater than 62 Gy.[14, 33, 34]
Recent data support the concept of subtotal resection followed by radiation therapy as an effective therapy equal in outcome to gross total resection but with fewer complications.
Preoperative workup includes an endocrinologic evaluation, particularly to exclude hypoadrenalism and hypothyroidism, both of which increase surgical mortality rates. Dexamethasone may be started prior to surgery to decrease edema.
Successful transsphenoidal resection of a craniopharyngioma requires a generous removal of the contents of the sella turcica. Adequate suprasellar decompression occurs when the arachnoid membrane that surrounds the tumor descends into the operative field. Cerebrospinal fluid (CSF) leakage occurs if the arachnoid is disrupted.
The goal of a craniotomy is gross total removal of the tumor with preservation of the optic apparatus and pituitary stalk. Understanding the anatomy in this region is key to accomplishing this goal. The optic chiasm is nearly always elevated, and the pituitary stalk is usually displaced posteriorly. The stalk may be identified by the striate pattern of portal vessels along its surface. The lamina terminalis may need to be opened for access into the third ventricle. The goal of tumor removal must not outweigh the need for preservation of neural structures; therefore, leaving undisturbed a tumor that is densely adherent to the optic apparatus, anterior cerebral artery, or hypothalamus is advisable.
In general, because long-term tumor control is excellent with radiation therapy following subtotal tumor removal, a conservative approach to tumor resection is usually advised. In some patients, however, a good cleavage plane exists between tumor and brain, and a true complete resection can be accomplished. Surgical judgment is crucial in the assessment of this possibility.
Performing a full postoperative endocrine evaluation as well as repeated imaging studies is advisable. MRI scans are typically obtained immediately postoperatively (within 48 h), at approximately 3 months, and annually thereafter. Long-term follow-up also includes visual-field and pituitary hormone testing.
The most common complications are related to injury of the adjacent neural structures. Vision may worsen because of unavoidable optic apparatus manipulation.
Postoperatively, many patients demonstrate hypopituitarism. In order to offset this, they are usually given physiologic doses of hydrocortisone as well as a dexamethasone taper to decrease the edema associated with the surgical approach.
Diabetes insipidus may develop and may require treatment with fluid replacement and, occasionally, vasopressin or its synthetic analogue, desmopressin. Some patients have diabetes insipidus as well as disruption of their thirst sensation. These patients pose a difficult management problem and are at high risk for developing hypernatremia. Injuries to the hypothalamus can cause other behavioral changes, including caloric balance disturbance, memory disturbance, and changes in affective behavior.
In rare cases, development of radiation-induced gliomas may occur. This long-term complication has a latency period of greater than 10 years, frequently involves the temporal lobe, and has been predominantly reported in patients who underwent conventional fractionated radiotherapy in their childhood.
Outcome and Prognosis
Total resection is the best chance for cure, although some series have reported good results with subtotal resection and fractionated radiation therapy. Small residual tumor, confirmed on postoperative MRI, is generally treated with external beam radiotherapy; however, stereotactic radiosurgery has been used. The use of proton beam radiotherapy for residual disease is currently being investigated. Although the amount of radiation is limited by the proximity to the optic chiasm, good long-term results are now being reported after radiosurgery.[40, 41, 42, 43, 44]
Adverse effects of radiation therapy include endocrine dysfunction, optic neuritis, dementia, and radiation necrosis. In addition, radiation can induce tumors such as meningiomas, sarcomas, and gliomas. In pediatric cases, radiation is postponed to minimize its effects on intelligence quotient and growth. Survival rates for patients with surgery and radiation are better than with surgery alone because radiation helps deter regrowth when residual tumor is present.
Brachytherapy has also been used to treat cystic craniopharyngiomas. Radioisotopes are placed into the cystic portions of the craniopharyngioma. Phosphorus-32 (32 P), colloidal gold-198, colloidal yttrium-91, and bleomycin have all been used. Bleomycin causes shrinkage of the cyst but is highly toxic to neural structures.
In a review of brachytherapy for craniopharyngiomas, Van den Berge followed 31 patients for an average of 41 months. Twenty-nine percent showed improvement of visual acuity, while 13% had stable visual acuity, and 58% deteriorated. Similarly, 28% showed improvement of their visual fields, with 20% showing no change, and 52% deteriorating.
Mortality and morbidity
Overall, the surgical mortality rate is less than 5%, mostly from hypothalamic injury. Bilateral hypothalamic injuries lead to hyperthermia and somnolence. The 10-year survival rate, excluding non–tumor-related deaths, is 90%.
The recurrence of craniopharyngiomas is reported to be up to 24% with approximately a 7-year follow-up. The main risk factor for recurrence is the presence of the residual tumor. Tumor recurrence is higher in the first 3 years after surgery.
In patients with gross total removal of a craniopharyngioma, the 5-year recurrence-free rate is 84.9%, compared to 48.3% in patients with subtotal removal.[47, 48] Morbidity and mortality rates are higher with recurrence and mainly present as visual deficits, endocrine abnormalities, hypothalamic injury, and neurocognitive and neurobehavioral deficits. The visual impairment can be caused by direct damage during surgery or from a daily dose of radiation greater than 2 Gy. The cumulative probability for visual deficits after surgery/radiation is reported to be 36-48% with a 10-year follow-up[48, 49] .
Endocrine abnormalities are commonly seen in postoperative patients with craniopharyngioma. It presents as hypopituitarism and is reported with deficiencies of at least 3 pituitary hormones in 54-100%.[48, 49] Preoperative endocrine deficits do not improve after the surgery, although patients with diabetes insipidus may improve. Hypothalamic dysfunction after the surgery may present as obesity because of hyperphagia, water balance impairment, loss of temperature control, sleep disorders, and neurocognitive disorders. Hypothalamic damage may result from tumor invasion, direct surgical damage from the resection of adherent tumor, tumor recurrence, and radiation.[48, 50]
Hoffman followed 50 children with craniopharyngiomas. In his 1992 report, 90% had total excision, and tumor recurred in 34%. Of all 50 children, 56% were leading normal or nearly normal lives, often requiring endocrine replacement. Twenty-four percent were able to function reasonably well and to attend school despite intellectual, visual, or weight problems; 8% were significantly handicapped; and 6% died. Effenterre et al reported that failure to achieve an independent living function with poor integration and performance at work or school is seen in 16% of adults and 26% of children.[48, 51] The data from literature show the importance of preoperative planning with a reasonable decision in terms of maximal safe tumor resection and the avoidance of complications.
Future and Controversies
The largest divide in the treatment of craniopharyngiomas regards whether to perform a gross total excision or perform a subtotal resection followed by radiation therapy. Retrospective series support both philosophies. Surgical judgment must temper the enthusiasm for gross total removal at all costs. The use of 3-dimensional conformal radiation treatment (3D CRT), stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT), and intensity-modulated radiation therapy (IMRT) will further allow treatment of small tumor residua with little risk of neurologic deficit. The development of endoscopic techniques has placed greater emphasis on minimally invasive approaches, but this should not detract from surgical techniques that the surgeon feels most comfortable using.
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