Neurosurgical Consultants: Nervous System Tumors

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Martin L. Lazar, MD, FACS, FAANS
James E. Bland, MD, FAANS

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RATHKE'S CYST TUMOR

Rathke's Cyst Tumors occur within the Sella Turcica adjacent to the Pituitary Gland which they then compress as the Cyst grows larger. The cells of the Cyst wall secrete a fluid substance, which as it slowly accumulates, progressively distorts the Pituitary Gland and can extend above the Sella Turcica to compress the visual sensory apparatus.

ETIOLOGY

These "tumors" result from some cells that were destined in embryological development of this patient to form part of the nasopharynx, to be trapped when they failed to migrate to their more appropriate destination.

SYMPTOMS

The most common symptoms exhibited by patients with Rathke's Cyst Tumor are:

Headache
Visual Sensory impairment

Pituitary dysfunction consequent to compression resulting in general tiredness

DIAGNOSIS

MRI Scan is the most reliable neuroimaging study for Rathke's Cyst Tumor although CT Scan is commonly used by primary physicians who are investigating their patient's complaint of headache. MRI scan findings are reasonably reliable although their can be confusion with a partially cystic Pituitary Tumor.

Figure 1A (Left): MRI Scan (Un-enhanced-T1 Weighted Image-Coronal View) of a Large Right-sided Rathke's Cyst in a 34 year old Male with headaches and mild Pituitary insufficiency. The tumor (Quadrangular Arrows) abuts the Right Carotid Artery (Horizontal Arrow indicating the round "Black" structure) and slightly displaces the Pituitary Stalk (Curved Arrow) towards the Left as it compresses the Pituitary Gland within the Pituitary Fossa.

Figure 1B (Right): MRI Scan (Gadolinium Enhanced Coronal View). Rathke's Cyst Tumor of the same patient as depicted in Figures 1A, 2A & 2B. The Pituitary Gland "enhances" (Curved Arrow) on this study. Compare to Figure 1A.

Figure 2A (Left): MRI Scan (Axial View-T2 weighted Image). Large Rathke's Cyst (Curved Arrow) eccentrically located to the Right side.

Figure 2B (Right): MRI Scan (Sagittal View). Rathke's Cyst (Up-curved Arrow) appears to fill the Pituitary Fossa (Sella Turcica) without displacing the Right Optic Nerve (Down-curved Arrow).

NOTE: This patient underwent a Trans-sphenoidal Resection of the Rathke's Cyst tumor in early 2000 and has remained free of symptoms and is followed with yearly MRI Scans.

TREATMENT

Surgery

The treatment of choice for patients with Rathke's Cyst Tumors is surgery. Most experienced surgeons recognize that there is a fairly high risk to recurrence, since it can be very difficult to remove the entire capsule. Transsphenoidal Microsurgery is the standard therapeutic approach for these lesions. Controversy remains among Neurosurgeons regarding the degree of vigor with which the surgeon pursues complete resection of the capsule since; this aggressiveness may result in permanent damage to the Pituitary Gland and its function. Recurrences are generally managed with repeat surgery.