Craniopharyngiomas are rare brain tumors that originate in the anterior superior margin of the pituitary gland. They account for 1%-3% of all intracranial tumors and have a calculated incidence of 338 cases per year in the United States (Bunin, et al., 1998). Although they are histologically benign, the overall treatment regimen for craniopharyngiomas is a challenge. Craniopharyngiomas have a tendency to adhere to vital brain structures, primarily the hypothalamus and pituitary gland; this factor makes total resection or cure difficult (Chakrabarti, Amar, Couldwell, & Weiss, 2005). Craniopharyngiomas often recur because they cannot be totally resected.
Effective treatment for patients with a craniopharyngioma requires communication and coordination among the multidisciplinary team (Vance, 2003). Nurses play a major role on the multidisciplinary team. This article reviews the pathophysiology, diagnosis, and treatment as well as the nursing implications of craniopharyngiomas. The emphasis is on effective communication and coordination of care.
The pituitary gland is a pea-size organ below the hypothalamus and within the sella turcica. It is divided into the anterior lobe and the posterior lobe. The sella turcica is in close proximity to delicate vascular and neurologic structures, including the optic chiasm and cavernous sinus, as well as the third, fourth, and sixth cranial nerves and the ophthalmic and maxillary division of the fifth cranial nerve (Fig. 1). An enlarging pituitary mass, such as a craniopharyngioma, can affect any of these structures (Martin, 2005).
[FIGURE 1 OMITTED]
The pituitary stalk, or infundibulum, connects the pituitary gland to the hypothalamus. It contains important nerve fibers (the hypothalamo-hypophysial tract) and small blood vessels (the hypothalamo-pituitary portal vessels) that carry hormones into the posterior and anterior pituitary lobes respectively (Vander, Sherman, & Luciano, 1994). Compression of the pituitary stalk by a craniopharyngioma can affect these nerve fibers and blood vessels and produce endocrine abnormalities.
Craniopharyngiomas are embryonic remnants of Rathke's pouch. They grow slowly from birth. Tumors can vary in size from a small, solid, well-circumscribed mass to a huge multilocular cyst. Most tumor cysts are filled with cholesterol crystals (Greenberg, 2001). A craniopharyngioma can develop in the pituitary stalk in the suprasellar region, next to the optic chiasm (Recht, 2005), and invade the sella turcica, shifting important neurologic structures around it. A craniopharyngioma can extend along the path of least resistance, laterally, medially, superiorly, or toward the base of the brain, shifting the third ventricle superiorly.
The molecular and biological bases of craniopharyngiomas are poorly understood. Remnant cells may lie dormant for decades before forming into a tumor (Ullrich, Scott, & Pomeroy, 2005).
Craniopharyngiomas occur equally among males and females. The common age groups for craniopharyngiomas are children 5-14 years of age and adults 50-74 years of age, but they can occur at any age (Bunin et al., 1998).
Signs and Symptoms
The hallmarks of a patient with a craniopharyngioma are vision loss, endocrinologic disturbances, and headache (Ullrich et al., 2005). Most craniopharyngioma patients seek treatment because they are experiencing visual deficits (Chakrabarti et al., 2005; Fahlbusch, Honegger, Werner, Huk, & Buchfelder, 1999; Van Effenterre & Boch, 2002). Optic chiasm compression can result in bitemporal hemianopia. Extraocular palsies may also occur as a result of tumor extension into the cavernous sinus. Headaches can result from distension of the diaphragma sellea or cerebrospinal fluid (CSF) obstruction, leading to hydrocephalus.
Signs and symptoms of endocrine dysfunction or panhypopituitarism may be manifested at the time of diagnosis. They include diabetes insipidus (DI), weight gain, menstrual irregularity or amenorrhea, hypothyroidism, growth failure (in children), and delayed or precocious puberty. These symptoms can also be related to hypothalamic or pituitary damage.
Craniopharyngioma patients are more likely to present with psychiatric symptoms, which may be characterized by loss of short-term memory, personality changes, dementia, and depression, compared with those diagnosed with other pituitary lesions (e.g., Rathke's cleft cyst, arachnoid cyst; Shin, Asa, Woodhouse, Smyth, & Ezzat, 1999). It is important to recognize that psychiatric symptoms are usually accompanied by neurological symptoms, such as headaches and visual deficits.
MRI (with and without contrast) and CT scanning are the prime neurological imaging techniques for diagnosing craniopharyngiomas. MRIs identify the extent of a craniopharyngioma and approximation to the third ventricle, but CT scans are best for identifying calcification (Freda & Post, 1999). MRIs also show whether the lesion has invaded the sella turcica, whether the lesion displaces the chiasm, and whether it invades the hypothalamus, as well as the location of adjacent major blood vessels.
The MRI appearance of craniopharyngiomas can vary depending on the proportion of solid versus cystic components, the amount of calcification, and the cystic fluid contents of the tumor. The solid components typically appear isointense or hypointense on T1-weighted images (Fig. 2) while hyperintense on T2-weighted images. Cystic components usually demonstrate a high signal on T1-weighted images due to high protein content (Freda & Post, 1999).
[FIGURE 2 OMITTED]
As with other pituitary-region tumors, ophthalmological and endocrine testing is required. Ophthalmological testing documents the extent of visual dysfunction and serves as a baseline for comparison after surgery. The visual examination includes evaluation of the visual field, visual acuity, and extraocular movements. Visual field testing ascertains whether the optic pathways are compressed.
Endocrine testing is crucial because most craniopharyngioma patients have hypopituitarism. Panhypopituitarism may include hypothyroidism, adrenal insufficiency, hypogonadism, growth hormone deficiency, and DI. Endocrine function should be evaluated to lessen complications during and after surgery (Vance, 2003). The patient should be referred for an endocrinology consultation before surgery for appropriate clinical testing and treatment. The endocrine blood tests include analysis of prolactin with dilution, insulin-like growth factor-I, adrenocorticotropic hormone, 8 am cortisol level, free thyroxine (FT4), thyroid-stimulating hormone, luteinizing hormone, follicle stimulating hormone, and testosterone level (for male patients; Vance, 2003).
Surgery is the primary treatment option for symptomatic craniopharyngioma patients. Surgery confirms the diagnosis and removes as much of the tumor as possible. Surgical goals include improving visual function and minimizing the worsening of endocrine function and neuropsychological impairment (Chakrabarti et al., 2005; Van Effentere & Boch, 2002).
Yasargil et al. (1990) described several approaches for the surgical treatment of craniopharyngioma: transsphenoidal, pterional, transcallosal, combined pterional-transcallosal, and combined pterional-transsphenoidal. The surgical approach chosen should be based on the patient's age and medical condition; the preference of the surgeon and patient; the experience of the surgeon; the tumor size, location, and extension; and the postoperative radiotherapy options available (Chakrabarti et al., 2005).
Greenberg (2001) recommended a transsphenoidal approach as the optimal surgical procedure because it has several advantages: no external scarring, no brain retraction, and, most likely, a shorter hospital stay. A transcranial approach or a craniotomy is indicated when the tumor extends beyond the boundaries of the sella turcica, when an unrelated pathology such as a parasellar aneurysm is suspected, or when the neurosurgeon suspects a fibrous tumor that cannot be completely removed by the transsphenoidal approach. Modern neuroimaging with CT or MRI allows the neurosurgeon to choose the precise surgical approach.
Unlike most benign tumors, craniopharyngiomas tend to recur and behave in a malignant manner. Complete resection may not be achieved for several possible reasons: the tumor's firm adherence to the hypothalamus, an obstructed view of the tumor, calcification, the tumor's adherence to perforating vessels, severe bradycardia during dissection, older age, significant blood loss when an aneurysm is present, or the tumor's very thin capsule. Even when complete tumor removal appears to have been achieved, a residual tumor may appear on an MRI after surgery (Fahlbusch et al., 1999).
Fractionated stereotactic radiotherapy (FSRT) is usually recommended when complete resection is not achieved during surgery or when the tumor recurs. Stereotactic radiosurgery (SRS) or FSRT increasingly is being utilized for both adult and pediatric brain tumors, including craniopharyngiomas (Kalapurakal, 2005). Both SRS and FSRT utilize the principle of stereotaxis, which allows precise target or tumor localization and accurate delivery of radiation to the tumor, either with a single session (SRS) or a series of sessions (FSRT), while reducing radiation dose to critical nearby structures (Kalapurakal, 2005). Both SRS and FSRT are noninvasive and can be performed on an outpatient basis without general anesthesia. However, SRS or FSRT should be used only after surgery to prevent or treat a recurrence (Laws & Vance, 1999).
Radioisotopes or chemotherapeutic agents, such as bleomycin, may be instilled, especially when treating residual or recurrent craniopharyngiomas (Amendola, Wolf, Coy, & Amendola, 2003). Bleomycin is an antineoplastic agent that stops the production of cystic fluid, causing tumor cells in the craniopharyngioma to shrink. Intracystic administration of bleomycin via an Ommaya reservoir, followed by radiation, is an alternative method for eradicating cystic craniopharyngiomas.
Prognosis and Outcomes
The survival rate for craniopharyngioma patients is excellent; 90% of all patients are still living 10 years after diagnosis (Ullrich et al., 2005). However, functional outcomes, quality of life, and community reintegration are of more concern for craniopharyngioma patients.
In a retrospective study performed by Duff et al. (2000), 73 of the 121 patients evaluated had a good outcome, with a mean follow-up period of 10 years. Good outcomes were associated with survival at the follow-up examination; the absence of major motor deficits related to treatment or tumor progression; functional vision; a Katz grade of A, which denotes independence and not requiring any supervision in feeding, continence, transferring, using the toilet, dressing, or bathing; school status at no more than 1 year behind the expected grade or employability for an adult of working age; the absence of incapacitating psychological or emotional problems; and a Karnofsky Performance Scale (KPS) score of at least 80. The KPS is an assessment tool for evaluating functional status or impairment that is widely used by clinicians. It consists of an 11-item rating scale, with scores that range from 100 (normal)unction) to 0 (nonfunctional or dead; Hickey, 2003).
Of the 73 patients who had a good outcome, 48 patients had a gross total resection of the tumor, and 25 patients had a subtotal tumor resection. Duff et al. (2000) concluded that gross total resection was associated with better clinical outcomes, compared with subtotal resection. Partial or subtotal resection was associated with tumor recurrence at 12.8% in the first year after surgery and 23.4% in the fifth year after surgery. Tumors located primarily in the sella turcica and tumors initially treated by the transsphenoidal approach were associated with good outcomes. Patients who were lethargic; exhibited visual deterioration, hemianopia or papilledema, tumor calcification or hydrocephalus, and tumor adhesiveness to surrounding neurovascular structures; and had been treated with a transcranial surgical approach were more likely to have poor outcomes (Duff et al.).
Chakrabarti et al. (2005) and Fahlbusch et al. (1999) agreed with Duff et al. (2000). Patients treated with a transsphenoidal approach had better visual outcomes compared with those who were treated transcranially. Chakrabarti et al. attributed this to the superior capability of rapidly decompressing the visual apparatus when employing the transsphenoidal approach.
Endocrinological outcomes may be unclear when endocrinological evaluations are not performed before surgery as can occur when emergency surgery is required. In a study performed by Honegger, Buchfelder, and Fahlbusch (1999), 143 patients underwent endocrinological testing before and after surgery. Ninety-two patients had a transcranial approach, while 35 patients had a transsphenoidal approach. (The remaining 16 patients underwent limited procedures such as stereotactic-guided cyst puncture or CSF shunt placement.) Panhypopituitarism increased from 10.9% before surgery to 34.8% after surgery for those treated transcranially; the increase was 40%-42.9% for those treated via the transsphenoidal approach. Honegger et al. concluded that there was no significant difference in the DI occurrence between the two groups. Regardless of the surgical approach, DI is an expected consequence of craniopharyngioma resection.
Craniopharyngioma patients may require hormone replacement before and after surgery as a result of injury to the hypothalamus or the pituitary stalk from the tumor or surgery. An endocrine service consultation is imperative both before and after surgery, to evaluate patients who may need lifelong hormone replacement therapy. Patients who are hormone deficient must have hormones replaced to physiological levels.
Adrenal insufficiency is treated with hydrocortisone (20 mg in the morning and 10 mg no later than 4 pm to replicate diurnal rhythms). A stress dose of glucocorticoid (usually a doubling of the current dose, for several days) is recommended during periods of acute infection, surgery, or physiological or psychological stress. Nurses and other healthcare providers must emphasize the need for stress-dose steroids. A patient's failure to receive adequate amounts of cortisone during stress can cause devastating metabolic complications, including circulatory collapse and death.
Patients receiving cortisone replacement therapy should be observed for signs of both undermedication (addisonian crisis), and overmedication (cushingoid symptoms). Signs and symptoms of addisonian crisis are weakness, dizziness, orthostatic hypotension, abdominal pain and cramping, and sodium and water retention. Cushingoid signs and symptoms include moon face, fat pads, a buffalo hump, acne, hirsutism, weight gain, psychic disturbances, peptic ulcers, and headache. Nurses should immediately report any of these symptoms to the primary care provider.
Patients undergoing cortisone replacement therapy should be observed for gastric irritation and tarry stools. Gastric irritation can be avoided by ingesting an antacid or a proton pump inhibitor with each medication. Nurses should warn their patients to not abruptly stop cortisone replacement therapy and tell their patients that gastric irritation or tarry stools must be reported immediately to their healthcare provider.
Hypothyroidism is treated with thyroid hormone replacement therapy, such as levothyroxine sodium (Synthroid). The dose should be adjusted in accordance with the free thyroxine (FT4) level. Serum thyroid-stimulating hormone usually is in the normal range in the setting of secondary hypothyroidism (Vance, 2003).
The need for sex hormones should also be evaluated after surgery. Women who are premenopausal should resume their menstrual cycles within a few months after surgery If menstruation does not occur, cyclical estrogen and progesterone are recommended until menopause is reached. Testosterone replacement is indicated for men who report a decreased libido or erectile dysfunction and have a low testosterone level. Men older than 40 years of age should have a prostate-specific antigen test before starting testosterone replacement. Both men and women dealing with fertility issues should be referred to a fertility center for possible gonadotropin treatment (Levy, 2004).
Craniopharyngioma patients should be evaluated for growth hormone deficiency. Growth hormone replacement therapy may be helpful in improving body composition by increasing muscle mass and reducing adipose mass. It may also help to improve muscle strength, exercise endurance, and serum lipid levels (Vance, 2003).
Central DI is the inability of the pituitary gland to secrete adequate amounts of antidiuretic hormone (ADH). This inability could result from injury to the hypothalamus, the supraoptic hypophyseal tract, or the posterior pituitary gland (Hickey, 2003). Nurses should be vigilant in watching for early DI signs and symptoms including polyuria, a urine output of 4-10 liters per day or an hourly output more than 200 cc for 2 consecutive hours or more than 3 cc per kg per hour in pediatric patients; extreme thirst in conscious patients; low urine-specific gravity (1.001-1.005); high serum osmolality; hypovolemia; signs of dehydration; and hypernatremia.
The management of patients at risk for DI include recording intake and output every hour; observing for signs of dehydration, such as tachycardia, hypotension, or orthostasis; performing neurological assessment, with particular attention to lethargy or change in mental status; and checking urine-specific gravity, serum osmolality, and electrolytes, as ordered. Any abnormalities should be reported immediately.
Depending on the degree of damage to the pituitary stalk, hypothalamus, or posterior pituitary gland, DI may be transient or permanent. Transient DI occurs in approximately 12% of patients who undergo pituitary surgery; 3% may have permanent DI after pituitary surgery (Vance, 2003). Craniopharyngioma patients have a very high risk of developing permanent DI.
Patients with transient DI may not require medical treatment. Patients who have an intact thirst mechanism and no functional impairment are usually capable of maintaining an adequate fluid intake. On the other hand, patients who are lethargic, are functionally impaired (e.g., unable to hold a glass or a cup), or have dysphagia may require intravenous fluid replacement to correct fluid imbalances.
Patients who cannot manage fluid losses with intake or have permanent DI require ADH replacement therapy. Intravenous vasopressin is recommended for patients with acute DI because of its immediate response and shorter action, which makes titration or adjustment easy. For patients who are alert enough to ingest or inhale their medications appropriately, oral or nasal desmopressin 1-deamino-8-D-arginine vasopressin (DDAVP) may also be administered. DDAVP dosing is patient-dependent; dose and frequency are individualized to a patient's needs. Patient condition is assessed by asking whether there is abnormal urinary frequency and thirst or observing for increased urinary output. Such information is helpful in properly adjusting DDAVP doses to maintain fluid balances.
The Potential for CSF Leak
A CSF leak is a potential complication after transsphenoidal surgery. It can occur several days or weeks after surgery. The leak is caused by an opening in the dura that allows fluid to drain through the nose. According to Sudhakar, Ray, and Vafidis (2004), the incidence of CSF leak after transsphenoidal surgery is 1.5%- 4.2%.
Nurses should observe the mustache dressing for clear, watery nasal drainage and look for any characteristics that may indicate the presence of a CSF leak. Patients and family members should also be instructed to immediately report any nasal drainage. Another characteristic of a CSF leak is a salty or sweet taste in the patient's mouth. Patients may develop a low-pressure headache when standing (Greenberg, 2001).
According to Kerr, Chu, and Bayles (2005), most CSF leaks heal spontaneously within 7-10 days. Conservative treatments include cautioning patients not to blow their nose and avoid sneezing, coughing, or any activities that stimulate straining (Hickey, 2003). Bed rest, with the head of bed (HOB) at 30[degrees] for several days, is also beneficial.
A lumbar drain may be inserted to allow healing at the operative site by decreasing CSF volume and pressure. A lumbar drain is usually inserted if a CSF leak persists with bed rest or if CSF is seen in the surgical field. Nurses should tell patients and families to ask for assistance in closing the lumbar drainage system before standing and before any other position change or ambulation, so as to prevent overdrainage headaches and accidental disconnection of the lumbar drain system. Assessing for changes in mentation and watching for signs and symptoms of meningitis are also indicated.
Late Effects of Radiotherapy
Patients who undergo pituitary radiation should be followed regularly for the possible development of new pituitary deficiencies (Vance, 2005). The effects of panhypopituitarism after radiation therapy may not be immediately evident, and deficiency may not become evident for several months or years. A baseline ophthalmological test and subsequent testing are required, because pituitary radiation can cause visual deterioration.
Complications such as radiation necrosis, cognitive decline, and the development of secondary malignancies occur in 2% of patients (Schulz-Ertner et al., 2002). Radiation-induced vasculopathy, such as moya moya disease, can occur as much as 6-12 years after radiation therapy (Kalapurakal, 2005).
Craniopharyngioma patients may develop pathologic obesity. It is more common among children diagnosed with a craniopharyngioma and occurs as a result of injury to the ventromedial hypothalamus, which is the area responsible for regulation of appetite and satiety. Patients tend to gain a profound amount of weight, which predisposes them to develop cardiovascular risks, such as diabetes mellitus and metabolic syndrome. Nurses and other multidisciplinary teams should refer patients to their primary care provider for early recognition and treatment of these cardiovascular risks, which can affect survival and the quality of life. Unfortunately, diet and exercise may be insufficient to control the pathologic obesity associated with craniopharyngioma. Further studies are needed to determine the most effective treatment.
Nurses and all team members have an important responsibility in educating craniopharyngioma patients and their families. The malignant nature of this tumor is often misunderstood. Patients and families who are well-educated about the importance of rigorous long-term follow-up tend to be more compliant and supportive of the care required. The need for serial MRIs, because of the high incidence of recurrence, should be emphasized. Patients and families must understand the importance of following up with several healthcare specialists in addition to the primary care provider. Often, patients and their families find the multitude of specialist appointments to be overwhelming. The side effects of medications, the importance of taking hormone replacement therapy and not stopping it unless instructed by the endocrinologist, the need for adjusting hormone replacement in case of any acute illness, and the need for periodic visual examinations are critical subjects to discuss with patients and families.
G. A., a high school senior, is a 17-year-old Hispanic male who was in his usual state of good health until 2 months before seeking medical attention from his primary care physician. At that time, G. A. noted decreased vision in his left eye; his vision seemed blurry when he looked to the left. He described trouble seeing things toward his left side. Although he had been wearing eyeglasses for 5 years, there had been a recent and acute decrease in his visual acuity. This complaint prompted a referral to an optometrist, who subsequently referred him to an ophthalmologist. Visual testing was performed, and a bitemporal hemianopia was identified. His left visual acuity was 20/200, while his right visual acuity was 20/20. The ophthalmologist suspected a pituitary tumor. An MRI with and without gadolinium was ordered; it revealed a pituitary mass hyperintense to the brain on T1-weighted images (Fig. 2) and isointense to the brain on T2-weighted images. The mass had a dumbbell shape and was centered in the sella turcica but extended into the suprasellar cistern and compressed the optic chiasm. G. A. was referred to a neurosurgery clinic for further evaluation.
After visiting the neurosurgeon, G. A. was referred to the endocrinology clinic for baseline endocrinological testing. On further history review, G. A.'s energy level was adequate and his bowel movements were regular. G. A. had gained about 50 lb (22.68 kg) during the last 3-4 months. He complained of headaches, which occurred primarily in the morning. He was urinating 2-3 times per night. His initial endocrine workup results were normal, however, including his sodium level.
His physical examination was normal except for his weight and visual deficits. No extraocular palsies were noted, but he had decreased visual fields with bitemporal homonymous hemianopia.
G. A. was admitted for a transsphenoidal hypophysectomy and tolerated the surgery well. After surgery, he was transferred to the intensive care unit. He then began to void more than 250 ml of diluted urine per hour for several consecutive hours. His sodium levels began to rise, and he was quickly placed on a 0.45% hypotonic fluid regimen with close monitoring of his sodium levels. Intravenous DDAVP was added because his sodium levels and urine output continued to be high despite the hypotonic fluid replacement. An endocrinology consultation was obtained for further recommendations on vasopressin and fluid replacement. Serum osmolality, urine-specific gravity, and sodium levels continued to be drawn and monitored every 6-8 hours. Accurate recordings of intake and output were reinforced.
On day 5, G. A.'s urine output slowly began to taper, until replacement fluids and DDAVP were no longer necessary. G. A. was able to sustain normal and stable sodium levels simply by drinking adequate amounts of fluids.
Immediately after surgery, G. A. had developed some lightheadedness, a salty taste in the back of his throat, and CSF rhinorrhea. A lumbar drain was inserted for 2 days to drain the CSF. The lumbar drain was then clamped for 24 hours and then removed because no further CSF rhinorrhea was noted.
On day 8, G. A. was neurologically stable and discharged to home. Hydrocortisone, DDAVP, and thyroid hormone replacement therapy were not considered necessary because his cortisol, sodium, and FT4 levels were adequate. G. A. and his family were educated throughout his hospital stay. They were scheduled for follow-up appointments with the endocrinology clinic; the neurosurgeon; and with the ear, nose, and throat specialist. G. A.'s vision had improved, but he was given a follow-up appointment with an ophthalmologist for a formal visual-field exam. A follow-up MRI was scheduled during his appointment with the neurosurgeon. The need to attend all appointments was reiterated.
Two months after surgery, an MRI showed an enhancing residual tumor in the sella turcica but no chiasmatic compression. G. A.'s endocrine lab results, including his sodium level, remained normal. He was referred to a radiation oncologist for possible radiation therapy. G. A. underwent FSRT for more than a month. He tolerated the entire radiation course well. He did not have any visual changes during the course of radiation treatment.
Treating patients with craniopharyngioma is a medical challenge for healthcare providers. Nurses are closest to the patient and are responsible for gathering and recognizing the subtle signs and symptoms associated with recurrence or possible complications of treatment. Overwhelming reactions can sometimes be observed among patients and families because long-term follow-up with several healthcare providers is required. Patient and family education plays a major role in adherence to craniopharyngioma treatment. The role of nurses in educating patients and families about the treatment for craniopharyngioma, and its complications, should not be underestimated.
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Questions or comments about this article may be directed to Margaret Alvarez, MSN RN APN, at firstname.lastname@example.org. She is an inpatient adult nurse practitioner in the Department of Neurosurgery at the University of Chicago Hospitals in Chicago.
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|Title Annotation:||care and treatment|
|Publication:||Journal of Neuroscience Nursing|
|Date:||Oct 1, 2006|
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