Craniopharyngioma

• Fred F. Ferri, M.D.

 Basic Information

Definition

Craniopharyngiomas are tumors arising from squamous cell remnants of Rathke’s pouch, located in the infundibulum or upper anterior hypophysis.

Synonyms

1. Subset of nonadenomatous pituitary tumors

ICD-10CM CODES
D44.3	Neoplasm of uncertain behavior of pituitary gland

Epidemiology & Demographics

Peak Incidence

Occurs at all ages; peak during the first two decades of life, with a second small peak occurring in the sixth decade.

Predominant Sex

1. •

   Both sexes are usually equally affected.

2. •

   Craniopharyngiomas are the most common nonglial tumors in children and account for 3% to 5% of all pediatric brain tumors.

Physical Findings & Clinical Presentation

1. •

   The typical onset is insidious and a 1- to 2-year history of slowly progressive symptoms is common.

2. •

   Presenting symptoms are usually related to the effects of a sella turcica mass. Approximately 75% of patients report headache and have visual disturbances.

3. •

   The usual visual defect is bitemporal hemianopsia. Optic nerve involvement with decreased visual acuity and scotomas and homonymous hemianopsia from optic tract involvement may also occur.

4. •

   Other symptoms include mental changes, nausea, vomiting, somnolence, or symptoms of pituitary failure. In adults, sexual dysfunction is the most common endocrine complaint, with impotence in men and primary or secondary amenorrhea in women. Diabetes insipidus is found in 25% of cases. In children, craniopharyngiomas may present with dwarfism.

5. •

   More than 70% of children at the time of diagnosis present with growth hormone deficiency, obstructive hydrocephalus, short-term memory deficits, and psychomotor slowing.

Etiology

Craniopharyngiomas are believed to arise from nests of squamous epithelial cells that are commonly found in the suprasellar area surrounding the pars tuberalis of the adult pituitary.

Diagnosis

Differential Diagnosis

1. •

   Pituitary adenoma.

2. •

   Empty sella syndrome.

3. •

   Pituitary failure of any cause.

4. •

   Primary brain tumors (e.g., meningiomas, astrocytomas).

5. •

   Metastatic brain tumors.

6. •

   Other brain tumors.

7. •

   Cerebral aneurysm.

Laboratory Tests

1. •

   Hypothyroidism (low FT₄, FT₃ with high thyroid-stimulating hormone).

2. •

   Hypercortisolism (low cortisol) with low adrenocorticotropic hormone.

3. •

   Low sex hormones (testosterone, estriol) with low follicle-stimulating hormone and luteinizing hormone.

4. •

   Diabetes insipidus (hypernatremia, low urine osmolarity, high plasma osmolarity).

5. •

   Prolactin may be normal or slightly elevated.

6. •

   Pituitary stimulation tests may be required in some cases.

Imaging Studies

1. •

   MRI (Fig. E1) or head CT if MRI is contraindicated. MRI features include a multicystic and solid enhancing suprasellar mass. Hydrocephalus may also be present if the mass is large. CT usually reveals intratumoral calcifications.

   

2. •

   Visual field testing for bitemporal hemianopsia.

3. •

   Skull film may show:

   1. 1.

      Enlarged or eroded sella turcica (50%)

   2. 2.

      Suprasellar calcification (50%)

Treatment

General Rx

1. •

   Traditionally, surgery has been the main treatment for craniopharyngioma. However, radiation treatment instead of surgery may be an option in selected cases.

2. •

   Surgical resection (curative or palliative).

   1. 1.

      Transsphenoidal surgery for small intrasellar tumors.

   2. 2.

      Subfrontal craniotomy for most patients.

3. •

   Overall prognosis is good with 80% to 90% chance of permanent cure.

4. •

   Postoperative radiation.

5. •

   Intralesional ³²P irradiation or bleomycin for unresectable tumors. Long-term complications of radiation include secondary malignancies, optic neuropathy, and vascular injury.

Prognosis

1. •

   Overall prognosis is good with 80% to 90% chance of permanent cure.

2. •

   Operative mortality rate: 3% to 16% (higher with large tumors).

3. •

   Postoperative recurrence rate: <20% of cases after total resection and 60% of cases after subtotal resection. Most recurrences occur within the first 2 yr after surgery.

4. •

   5-yr and 10-yr survival: 88% and 76%, respectively, with surgery and radiation.

5. •

   The most important factors that correlate with prognosis are the extent of resection and postoperative radiation.

6. •

   Long-term post-treatment hormonal, visual, and neurological problems occur in a significant percentage of patients.

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1. Craniopharyngioma (Patient Information)