Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

at initial treatment

1st line – surgical biopsy ± resection

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ACUTE
|
at initial treatment
1st line – 

surgical biopsy ± resection

Surgery is almost always performed, regardless of age, in order to obtain tissue for definitive diagnosis; relieve mass effect on surrounding neural structures; treat hydrocephalus.[2][11][14][28][29][30][31]

Surgery relieves raised intracranial pressure, if present (with or without cerebrospinal fluid [CSF] shunting), and may reverse visual compromise due to compression by tumour (improvement in nearly 40% of patients).

Surgery may include biopsy only, cyst aspiration, CSF shunting, or tumour resection (either partial or complete). The extent of surgical resection and the potential risks of surgery depend on tumour size and location.[10][14][29][33][34] 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.

The surgical approach depends upon the anatomy (relationship to the sella, optic chiasm, and third ventricle) and size of the tumour as determined by cranial magnetic resonance imaging.[27]

A number of surgical approaches may be used:

Transventricular (transcortical or transcallosal; from above) for tumours that involve the third ventricle and are above the optic chiasm

Sub-frontal and interhemispheric approaches (from underneath or between the frontal lobes) for tumours located anterior to the optic chiasm or just behind the lamina terminalis

Pterional approach (fronto-temporal window, through the opened Sylvian fissure) for small tumours in close proximity to the optic chiasm

Trans-sphenoidal approach (either transnasal or sub-labial) for partly or totally intrasellar (pituitary fossa) tumours

Endoscopic endonasal surgery is increasingly used (especially in children) for suprasellar as well as sellar tumours, due to a decreased risk of hypothalamic injury and higher chance of gross total resection.[35][36] It is typically trans-sphenoidal, with more expanded approaches used for larger craniopharyngiomas.

For multi-compartmental tumours, a combined approach using two or more of these options may be used.

Plus – endocrine replacement therapy

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ACUTE
|
at initial treatment
Plus – 

endocrine replacement therapy

Treatment recommended for ALL patients in selected patient group

Endocrinopathies require replacement therapy depending upon the specific endocrine deficiencies.[4][8][9][11][49][52][53]

No improvement is seen in 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 diabetes insipidus (>70% in children; 50% in adults).[4][8][9][49][52][53]

Plus – radiotherapy

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ACUTE
|
at initial treatment
|
surgical biopsy only or incomplete surgical resection
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Radiotherapy is reserved for patients who have had either surgical biopsy only, or incompletely resected tumours.[38][39][40][41]

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 tumour size, its anatomical relationship to surrounding neural structures (in particular, the optic chiasm and chiasm), and availability.

Conventional fractionated external-beam radiotherapy (photon-based) administered daily for 5 to 6 weeks with a median tumour dose of 54 Gy is considered the standard of care for most tumours.[40][41]

Stereotactic radiosurgery (Gamma Knife, or linear accelerator [LINAC]-based) is best suited for the treatment of small (<2 cm), spherical tumours, or low-volume residual disease, which is anatomically separate from the optic chiasm and nerve.[40][45][46][47][48]

ONGOING

post initial treatment

1st line – continued endocrine replacement therapy

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ONGOING
|
post initial treatment
1st line – 

continued endocrine replacement therapy

Endocrinopathies require continued replacement therapy depending upon the specific endocrine deficiencies.[4][8][9][11][49][52][53]

No improvement is seen in 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 diabetes insipidus (>70% in children; 50% in adults).[4][8][9][49][52][53]

Plus – surgery ± intracavitary chemotherapy/radiotherapy

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ONGOING
|
post initial treatment
|
recurrent/refractory tumours
Plus – 

surgery ± intracavitary chemotherapy/radiotherapy

Treatment recommended for ALL patients in selected patient group

Repeat surgery may be of benefit.[54] Radiotherapy may be used to treat recurrence, and observational data suggest that stereotactic radiosurgery is an effective, minimally invasive option in this context.[45]

Recalcitrant cystic tumours may require 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 or yttrium.[55][56][57]

Alternatively, chemotherapy agents may be placed; a variety of drugs have been utilised, including bleomycin, methotrexate, and cytarabine.[58] Caution is required if intracystic therapies are used, as leakage carries toxicity to the optic structures and perforator vessels.

Consider – systemic chemotherapy

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ONGOING
|
post initial treatment
|
recurrent/refractory tumours
Consider – 

systemic chemotherapy

Additional treatment recommended for SOME patients in selected patient group

Data for chemotherapy in refractory craniopharyngioma are limited, and there is no standard systemic regimen.

In general, chemotherapy is reserved for patients in whom tumour persists despite surgery/radiotherapy and there are no other therapeutic options.[54]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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