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 tumor (improvement in nearly 40% of patients).

Surgery may include biopsy only, cyst aspiration, CSF shunting, or tumor resection (either partial or complete). The extent of surgical resection and the potential risks of surgery depend on tumor size and location.[10][14][29][33][34] Gross total resection should be achieved if possible; however, while maximizing resection, long-term side effects should also be minimized, bearing in mind the availability of adjuvant radiation therapy for subtotally resected lesions.

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

A number of surgical approaches may be used:

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

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

Pterional approach (frontotemporal window, through the opened Sylvian fissure) for small tumors in close proximity to the optic chiasm

Transsphenoidal approach (either transnasal or sublabial) for partly or totally intrasellar (pituitary fossa) tumors

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

For multicompartmental tumors, 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 hyperprolactinemia). Indeed, the incidence increases following therapy as a consequence of surgery (lowest risk with transsphenoidal surgery).

Approximate prevalence of specific deficiencies includes growth hormone (75%), hypogonadotropic hypogonadism (75%), adrenocorticotropic hormone deficiency (25%), hypothyroidism (25%), and diabetes insipidus (>70% in children; 50% in adults).[4][8][9][49][52][53]

Plus – radiation therapy

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

radiation therapy

Treatment recommended for ALL patients in selected patient group

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

The radiation therapy 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 tumor size, its anatomic relationship to surrounding neural structures (in particular, the optic nerve and chiasm), and availability.

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

Stereotactic radiosurgery (Gamma Knife, or linear accelerator [LINAC]-based) is best suited for the treatment of small (<2 cm), spherical tumors, 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 hyperprolactinemia). Indeed, the incidence increases following therapy as a consequence of surgery (lowest risk with transsphenoidal surgery).

Approximate prevalence of specific deficiencies includes growth hormone (75%), hypogonadotropic hypogonadism (75%), adrenocorticotropic hormone deficiency (25%), hypothyroidism (25%), and diabetes insipidus (>70% in children; 50% in adults).[4][8][9][49][52][53]

Plus – surgery ± intracavitary chemotherapy/radiation therapy

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

surgery ± intracavitary chemotherapy/radiation therapy

Treatment recommended for ALL patients in selected patient group

Repeat surgery may be of benefit.[54] Radiation therapy 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 tumors may require surgical drainage/aspiration and sometimes placement of an intratumoral cyst catheter and subgaleal 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 utilized, 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 tumors
Consider – 

systemic chemotherapy

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 tumor persists despite surgery/radiation therapy 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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