Approach

Surgery is almost always performed, regardless of age, in order to:[3][12][21][31][32][33][34]

  • obtain tissue for definitive diagnosis

  • relieve mass effect on surrounding neural structures, or

  • treat hydrocephalus.

Surgery relieves raised intracranial pressure, and prevents progressive worsening of presenting symptoms or development of new symptoms.

Surgery may include biopsy only, cyst aspiration, or tumour resection (either partial or complete). Surgery can also include placement of a CSF diverting shunt to treat any associated hydrocephalus. This may follow placement of a temporary external ventricular drain for management of acute hydrocephalus. On occasion, high-dose intravenous corticosteroids can be used to treat any associated peri-tumoural oedema.

Contrast-enhanced brain imaging (magnetic resonance imaging [MRI]/computed tomography [CT]) helps determine the surgical approach and provides a baseline for evaluating residual disease and response to treatment.

Baseline endocrine and ophthalmological evaluations with appropriate consultations must be performed to aid in monitoring effects of surgery upon endocrine function.[7][9][10][25]​​ If high-dose intravenous corticosteroids are started for peri-tumoural oedema, they should be tapered to oral hydrocortisone or the patient evaluated for adrenal insufficiency once treatment with high-dose corticosteroids has been stopped.[35]

Following primary surgery, radiotherapy may be required for any residuals.[36] As endocrinopathies are common (both pre- and postoperatively), hormone replacement therapy is usually necessary, depending upon the specific endocrine deficiencies.[Figure caption and citation for the preceding image starts]: Craniopharyngioma: sagittal post-contrast MRIFrom the collection of Marc C. Chamberlain; used with permission [Citation ends].com.bmj.content.model.Caption@1e43ed11[Figure caption and citation for the preceding image starts]: Craniopharyngioma (postoperative): sagittal post-contrast MRIFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@75ff7200

Surgery

The extent of surgical resection and the potential risks of surgery depend on tumour size and location.[11][21][32][37][38]

Gross total resection should be achieved if possible; however, while maximising resection, long-term side effects should also be minimised, bearing in mind the availability of adjuvant radiotherapy for subtotally resected lesions.

When adherent to nearby structures, such as the basilar artery, hypothalamus, or optic chiasm, surgery may be limited. In these cases, tissue biopsy for diagnosis and decompression of important anatomical structures is often attempted, without complete resection. Caution is required if intracystic therapies are used, as leakage carries toxicity to the optic structures and perforator vessels.

Surgical approach

Depends upon the anatomy (relationship to the sella, optic chiasm, and third ventricle) and size of the tumour as determined preoperatively by cranial MRI.[29] A number of surgical approaches may be used:[39][40][41][42]

  • Transcortical or transcallosal approaches allow a transventricular (from above) approach, which is indicated for tumours that involve the third ventricle and are above the optic chiasm.

  • Sub-frontal translamina terminalis approach is indicated for tumours located anterior to the optic chiasm or in the anterior third ventricle.

  • Pterional trans-sylvian approach. It can be combined with an orbito-zygomatic approach and translamina terminalis depending on tumour extent and location.

  • Microscopic trans-sphenoidal approach (either transnasal or sub-labial) is utilised if the tumour is totally intrasellar (pituitary fossa).

  • Extended endoscopic endonasal surgery is increasingly used given the ability to directly visualise the tumour relationship to critical structures such as the optic chiasm, pituitary gland etc. It is associated with decreased risk of hypothalamic injury and higher chance of gross total resection.

For large multi-compartmental tumours, a combined approach using two or more of these options may be used either in combination or a staged fashion.[11][38]

Surgical complications include risk of arterial injury (anterior cerebral arteries, carotid and the basilar artery) with resulting strokes, pituitary dysfunction, arginine vasopressin deficiency (AVP-D; previously known as diabetes insipidus), visual loss, and hypothalamic dysfunction. A higher incidence of hormone deficiency has been reported among paediatric patients undergoing complete resection compared with those managed with conservative surgery followed by adjuvant radiotherapy.[43][44]

Radiotherapy

Radiotherapy is typically used in the treatment of patients who have had either surgical biopsy or incompletely resected tumours.[44][45][46][47]

Although complete resection remains the mainstay for select patients, data from retrospective studies suggest that long-term outcomes with subtotal resection followed by adjuvant radiotherapy continue to improve and that subtotal resection without adjuvant radiation was an independent risk factor for tumour recurrence/progression.[16][48][49]​​ The optimum timing of radiotherapy in children remains uncertain, and due to the cognitive risks of administering radiotherapy to young children, delaying until there is evidence of progression may be preferable in selected individuals.[26]

Advances in radiotherapy techniques have made this option more effective and safe, and definitive radiotherapy may be an option in some patients. Whilst this remains controversial, observational data suggest that outcomes with definitive radiotherapy (radiotherapy alone) are comparable to those of established craniopharyngioma treatment modalities.[50][51] Head-to-head prospective studies are required.

Modality

The radiotherapy technique used (conventional fractionated external beam radiation, conformal external beam radiation; or advanced techniques including intensity-modulated radiation, stereotactic radiosurgery, and proton beam therapy) is dependent upon patient and tumour factors including patient age and preference, prior treatment for craniopharyngioma, the size of the residual tumour, its anatomic relationship to surrounding neural structures (in particular, the optic nerve and chiasm), and availability. Conventional fractionated external-beam radiotherapy (photon-based) administered daily for 5-6 weeks with a median tumour dose of 54 Gy is considered the standard of care for most tumours.[46][47]​ Where available and suitable, precision modalities (e.g., proton beam therapy, fractionated stereotactic radiotherapy) seem to be associated with higher rates of tumour control.[52][53]

Stereotactic radiosurgery involves the delivery of a single large fraction, or several fractions, of radiation dosage, either via a linear accelerator (LINAC) or as multiple cobalt beams (Gamma Knife). Limited studies exist, although experience with other extra-axial intracranial tumours (e.g., meningiomas) suggests this is credible and effective therapy. The application of stereotactic radiosurgery is limited by tumour location in relation to the optic chiasm and nerve, and tumour size and geometry. The technique is best suited for the treatment of small (<2 cm), spherical tumours, or low-volume residual disease.[46][54][55][56][57]

Radiotherapy in many instances will result in hypopituitarism and consequently the need for hormone replacement therapy.[25][56][58] Other possible complications include loss of vision (secondary to a radiation-induced optic neuropathy), rarely radiation injury/necrosis of the anterior temporal lobes, radiation injury to the hypothalamus, and induction of neurocognitive deficits.[59][60]

Endocrine therapy

Endocrine replacement therapy depends upon the specific endocrine deficiency.[5][9][10][12][58][61][62] No improvement is seen in baseline endocrine dysfunction following surgery (with the possible exception of hyperprolactinaemia). Indeed, the incidence increases following therapy as a consequence of surgery (lowest risk with trans-sphenoidal surgery).

Approximate prevalence of specific deficiencies includes growth hormone (75%), hypogonadotrophic hypogonadism (75%), adrenocorticotrophic hormone deficiency (25%), hypothyroidism (25%), and AVP-D (>70% in children; 50% in adults).[5][9][10][61][62]

See Hypopituitarism.

Management of recurrent/refractory disease

Recurrent or treatment refractory disease is established both clinically and radiographically. If present, repeat surgery may be indicated.[49][63]​​​ Radiotherapy may be used to treat recurrence, and observational data suggest that stereotactic radiosurgery is an effective, minimally invasive option in this context.[54]

Recurrences may manifest as a recalcitrant tumour cyst requiring surgical drainage (aspiration) and sometimes placement of an intratumoural cyst catheter and sub-galeal reservoir. This allows repeated fluid aspiration and, if necessary, instillation of intracavitary radioactive colloidal phosphorus/yttrium.[64][65][66]​ Alternatively, chemotherapy agents may be placed; a variety of drugs have been utilised, including bleomycin, methotrexate, and cytarabine.[67] Intracystic chemotherapy may be considered in a research setting for persistent tumours despite other therapies, but it is not a first-line treatment option. 

Caution is required if intracystic therapies are used, as leakage carries toxicity to the optic structures and perforator vessels.

In exceptional instances (e.g., tumour still present despite prior surgery/radiotherapy and no longer operable), patients may be treated with systemic chemotherapy.[63] However, data for chemotherapy in refractory craniopharyngioma are limited, and there is no standard systemic regimen.

Long-term sequelae

In addition to immediate management of the craniopharyngioma and endocrine deficiencies, the detection and management of the potential longer-term sequelae (such as delayed optic atrophy, hypopituitarism, obesity, hyperlipidaemia, and diabetes mellitus) is essential, particularly in children.[58][68][69]

Quality of life is significantly impacted. Craniopharyngioma is best thought of as a chronic disease that is multi-dimensional.[70] Additionally, adults with craniopharyngioma have a substantially elevated risk of cardiovascular disease relative to the general population, added to the reduced health already common to these patients.[71]

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