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CRANIOPHARYNGIOMA

Craniopharyngiomas are intracranial Skull Base Tumors that are typically both cystic and solid in structure. They occur most commonly in childhood and adolescence and in adult life after age 50 years. They account for 2-4% of primary Brain tumors, 10-15% of Sellar and Suprasellar tumors (tumors that occur in and above the Pituitary Fossa also known as the "Sella Turcica") and 50-60% of Sellar and Suprasellar tumors, in children.

Craniopharyngiomas are usually not discovered until they impinge upon important structures around them, and are frequently quite large (over 3 cm), when detected. They are histologically benign (not malignant) tumors, but they have a tendency to become adherent to structures in and around the Pituitary Gland and Pituitary Stalk, including the Optic Nerves, Optic Chiasm, intracranial arteries and the Brain itself.

ETIOLOGY

Craniopharyngiomas are believed to arise from remnants of the Craniopharyngeal duct, or Rathke's pouch, which are developmental structures related to the primitive gut. Embryonic cells from an incompletely "involuted" Craniopharyngeal duct, or transformation of squamous cells in the lower infundibulum, or anterior Pituitary Gland, may give rise to a Craniopharyngioma. These tumors are thought to be closely related to another cystic mass, occasionally seen in the Pituitary region called a Rathke's Cleft Cyst.

SYMPTOMS

The symptoms produced by a Craniopharyngioma, vary depending upon the tumor's location. If it compresses the Pituitary Stalk, or involves the area of the Pituitary Gland itself, the tumor can cause partial, or complete Pituitary hormone deficiency. This frequently results in one or more of the following: growth failure, delayed puberty, loss of normal menstrual function or sexual desire, increased sensitivity to cold, fatigue, constipation, dry skin, nausea, low blood pressure, and depression.

Diabetes Insipidus, is a condition associated with the patient's production of massive volumes of urine and experiencing unquenchable thirst, both resulting from compression of the Pituitary Stalk resulting in a deficiency in the release of the Anti-diuretic Hormone (ADH). (See Pituitary Gland Tumors Patient Information for a discussion of Diabetes Insipidus also called "DI")

Additionally, Pituitary stalk compression can result in increase production of the hormone Prolactin, which causes breast milk production (galactohhrea).

Visual disturbances, of different degrees of severity (including blindness), can result if the Craniopharyngioma involves the Optic Tracts, Optic Chiasm, or Optic Nerves.

Hypothalamic disturbances can result from involvement of the Hypothalamus (an area in the very center of the Brain) which may cause in obesity, increased drowsiness, temperature regulation abnormalities, and diabetes insipidus.

Personality changes, headache, confusion, and vomiting are among the other symptoms manifested in these patients.

DIAGNOSIS

The best means of visualizing a Craniopharyngioma is with an MRI or a CT scan of the Pituitary region, performed with and without "contrast enhancing" Gadolinium.

TREATMENT

Surgery

Surgery is the preferential initial treatment for a Craniopharyngioma. The goal of surgery is to completely remove the tumor, while improving or at least preserving Pituitary, visual and Brain function.

Depending upon the location of the tumor, several approaches may be utilized.

  1. A Skull Base Tumor Craniotomy approach (See Figures 1-3) is generally required for optimal exposure and tumor removal if the tumor is predominantly above the Pituitary Fossa (Suprasellar).

  1. A Minimally Invasive Microendoscopic (or Microsurgical) Transsphenoidal technique can be used in a minority of patients in whom the tumor is relatively small (less than 3 cm) and located primarily within the Sella Turcica (the Pituitary Fossa).


Figure 4A (Top Left): Lateral Skull X-ray. Calcified Intrasellar Craniopharyngioma.

This 28 year old Male presented with Pituitary insufficiency. The calcified (Top Arrow) tumor lies within and slightly above the Sella. The MINIMALLY INVASIVE TRANSSPHENOIDAL APPROACH is conducted along a path indicated by the Lower Arrow.

Figure 4B (Top Right): Operative Photo (Same Patient) After the Anterior bone wall and the Dura of the Sella have been opened, the Tumor begins to extrude through the opening.

Figure 4C (Bottom Left): Operative Photo (Same Patient - after removal of the Tumor, looking into the interior of the Sella) The bone opening of the Sella is clearly seen. The Suprasellar Arachnoid has descended into the Sella (Vertical Arrows) and must be repositioned upwards with implanted muscle or fat (which is standard for this operation.)

Figure 4D (Bottom Right): Operative Photo (Same Patient) The Sella has been reconstructed and sealed using small pieces of cartilage obtained from the back of the Nasal Cartilage at the beginning of the operation by the ENT Surgeon.


Figure 5: Intrasellar Craniopharyngioma Specimen
After complete Minimally Invasive Transsphenoidal Removal (Same Patient as Figures 4A-4C).

The patient's Pituitary insufficiency required long-term Endocrinological management with supplemental hormones. He remains free of tumor.


Radiation Therapy

Radiation treatment frequently offers some degree of tumor control and prolonged survival in those patients where the tumor cannot be completely removed. Stereotactic Radiation is the preferred method of Radiation since, it more safely, delivers a higher radiation dose to the tumor although conventional (external beam) radiation therapy can be effective in controlling growth of Craniopharyngiomas as well.

TENDENCY TO RECUR and FOLLOW UP

Craniopharyngiomas have a tendency to recur, particularly if there has not been complete surgical removal. Long term follow up is required with repeat MRI's or CT scans. These are usually obtained once a year. The treatment team usually recommends the frequency and the length of time for follow up depending on the particular circumstances of each patient.

Endocrinological follow up is equally important, particularly for those patients who were treated with Radiation Therapy, since hormone deficiencies can develop many years after this treatment.


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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.