Patient With Craniopharyngioma

Patient With Craniopharyngioma

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A craniopharyngioma is a benign slow growing tumor most commonly occurring in the suprasellar region of the brain near the pituitary gland and optic nerves. The overall incidence of this tumor is about 1.2% and 4.6% with a peak incidence in children aged 5-14 years and in adults between 65 and 74 years of age (1, 2). The incidence does not vary by gender or race (2). These tumors account for 2% to 5% of all primary intracranial neoplasms (3).

Clinical Presentation. The most common presenting clinical features of craniopharyngiomas include headaches and visual field defects as well as nausea and vomiting (3, 4). Usually the visual disturbance manifests itself as a bitemporal hemianopsia as was the case with our patient. However, patients can also present with a homonymous hemianopia (5). The endocrine function may be suppressed and therefore manifest hypopituitarism, hypothyroidism, diabetes insipidus and or impotence (3, 4,5). Our presenting patient was positive for erectile dysfunction. On the other hand, there can also be an exaggeration of the endocrine system that may present as precocious puberty in children and obesity in adults (5).
Differential Diagnosis. The most common differential diagnosis considered when evaluating CT or MR images of craniopharyngiomas are (6):

Pituitary adenomas
Chiasmatic gliomas
Rathke’s pouch cyst
Epidermoid tumor

Pituitary adenomas tend to be bulkier and more likely to expand the sella, whereas craniopharyngiomas tend to expand upwardly leaving the bony sella unexpanded. The optic pathway gliomas differ from craniopharyngiomas in that they rarely involve the sella and are not usually calcified. Rathke’s cleft cysts can sometimes be challenging to differentiate from cranipharyngiomas, but these cysts are usually not solid or calcified. Epidermoid tumors rarely appear in the suprasellar area, but are still considered a possibility keeping in mind that enhancement of the peripheral rim is less common when compared to cranipharyngiomas (6).

Etiology. Craniopharyngiomas are not glial or neuronal lesions. They are epithelial neoplasms that can be both solid and cystic in nature (7). There are two theories as to how these tumors arise, the embryogenetic theory and metaplastic theory. The embryogenetic theory states that the Rathke’s pouch, which gives rise to the anterior pituitary gland during the fourth week of gestation, and the remnants of the craniopharygeal duct (the neck of the Rathke pouch) may be the site of origin for craniopharyngiomas. The metaplastic theory states residual squamous epithelial cells of the ectoderm of the embryo may undergo metaplasia and develop into a craniopharyngioma (5, 8).

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There are three major clinical syndromes associated with the specific location of a craniopharyngioma. A craniopharyngioma located before the chiasm usually results in optic atrophy and visual field constriction. A retrochiasmal mass is associated with hydrocephalus and signs of increased intracranial pressure. In this case the patient will more than likely complain of headaches as well as double vision, and the fundus exam may reveal papilledema. An intrasellar craniopharyngioma presents with headaches and a hormonal imbalance (8). Though craniopharyngiomas are benign histologically, they are aggressive in behavior because they have a tendency to affect surrounding structures, which include the visual apparatus, hypothalamus, pituitary stalk, 3rd ventricle and vasculature from the circle of Willis (1).
Diagnosis. In order to properly diagnose a craniopharyngioma, the work-up should include a neuro-ophthalmic evaluation, a visual field exam, as well as imaging and endocrinologic studies. The modality of choice when imaging a craniopharyngioma is magnetic resonance (6). This usually shows a T1 high intensity area which is usually due to high protein content in the fluid of the cyst (6) In regards to endocrinologic studies, the labs should include serum electrolytes, thyroid studies, cortisol levels, growth hormone levels, luteinizing and follicle stimulating hormone levels (8).
Treatment. The man who first introduced the term ‘cranipharyngioma’ in 1932 described it as “one of the most baffling problems which confront the neurogurseon” (5, 9). The mainstay treatment is microsurgical resection. There are two approaches to the surgical procedure, transcranial or transphenoidal (figure 8). In a study of 168 patients undergoing surgery for the treatment of craniopharyngioma, the transcranial approach was the most frequently used (10). The transphenoidal approach had a lower mortality rate when compared with the transcranial route. Total tumor removal was accomplished 85.7% of the time during the transphenoidal procedure and only 45.7% using the transcranial route. The selection of the surgical approach is determined by the location of the tumor (7). The other modality in treating these tumors in addition to surgery is external beam radiotherapy (11). This approach is mainly reserved for patients who are not fit for surgery or in the case of residual or recurrent craniopharyngiomas (7, 9). In a review of 10 major studies which included a total of 264 patients in which gamma knife stereostatic radiosurgery was used to treat craniopharyngiomas, tumor control was accomplished in 75% of the cases (12).

Figure 8 a) transcranial b) transphenoidal surgical approach (13)
In addition to external beam radiotherapy, there is another minimally invasive approach called cyst aspiration. This method is reserved for patients with craniopharyngiomas that are predominantly cystic composed (1).
Recurrence. An analysis of 121 patients with craniopharyngiomas presenting to the Department of Pediatrics and Endocrinology between 1964 and 2003 showed there was a significant difference in the recurrence-free survival rate depending on the surgical treatment modality (4). The records of the patients were divided into 6 different groups (groups A-F). Group A consisted of 16 patients who underwent gross total tumor removal, group B included 3 patients who had gross total removal with follow-up radiotherapy, group C included 51 patients who underwent partial removal only, group D consisted of 33 partial removals with follow-up radiotherapy, group E included 6 cyst evacuations alone and finally group F consisted of 3 cyst evacuations with follow-up radiotherapy. The recurrence-free survival rate results at 10 years were as follows: 100% (A), 100% (B), 38% (C), 77% (D). Re-accumulation of the cyst happened in 58% of the patients in group F and in none of the patients in group E (4). There are other predictors of recurrence besides the extent of resection such as tumor size and whether the tumor has extended into the third ventricle as well as the presence of hydrocephalus and calcification (9). A majority of patients with recurrent cranipharyngiomas are symptomatic, and the two most common presenting symptoms are visual disturbances and headaches (9).
Prognosis. The five-year survival rate is 85% in children and 40% in adults (5). Many of these patients end up with hypothalamic dysfunction, an altered neuropsychological state, and visual field defects (5, 14). In addition, there is a high long-term cardiovascular morbidity (7). Patients who have a recurrence are more likely to have worse functional outcome and 29% more likely to have a permanent neurological deficit after repeat surgery (9). Repeat surgery is also associated with higher mortality and morbidity (9).
A study of 54 patients who underwent surgery for craniopharyngiomas at the Leiden University Medical Center showed a high prevalence of long-term cardiovascular, neurological and psychosocial morbidity (15). At a median follow-up period of 10 year (ranging from 36 months to 37 years), 7 patients had suffered from cerebrovascular accident, 1 patient had a transient ischemic attack and 3 patients experience a myocardial infarction. The cardiovascular morbidity was more common in premenopausal estrogen-deficient women. Neurological morbidity included short-term memory loss in 21 patients, personality changes in 16 patients, specific brain function impairment in 14 patients, anosmia in 8 patients and position dependent vertigo in 6 patients. Twenty-five patients showed psychosocial morbidity, which included inability to resume work, impaired school performance and visual deficits (15). Ten patients died, 40% from cardiovascular complications and 30% from infections, one patient died from cerebral hemorrhage. Most of these deaths occurred years after the initial operation (15).

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