Abstract
The distinction among craniopharyngioma (CR), Rathke's cleft cyst (RCC), and intrasellar arachnoid cyst (AC) remains a difficult preoperative problem. Accurate diagnosis of these rare pituitary lesions is important to determine the type of treatment and predict prognostic outcome. The majority of the literature describes the clinical manifestations and management of only one of CR, RCC, or AC, rendering comparisons difficult. We conducted a study to 1) investigate distinguishing preoperative clinical, biochemical, and radiographic features of patients with CR, RCC, and AC; and 2) identify clinicopathological features that independently predict recurrence in CR and RCC in adults. Fifty-two adult patients included 21 patients with CR (mean age at initial surgery, 35 ± 14 yr), 26 patients with RCC (mean age, 37 ± 14 yr), and 5 patients with AC (mean age, 53 ± 12 yr). Mean follow-up duration was 70 ± 13 months. Patients with CR presented with hypopituitarism in 95% of cases and hyperprolactinemia in 38%. These patients also had more preoperative neurological deficits (67%), ophthalmological complaints (67%), and significantly higher psychiatric manifestations (33%; P = 0.003) than those with RCC or AC. Patients with AC presented with headaches (60%), visual field deficits (60%), or impotence (50%) in the absence of other specific endocrine dysfunction symptoms. Using biochemical criteria, the percentage of patients with two or more pituitary hormonal axes impaired preoperatively was 67% for CR and 62% for RCC, significantly greater (P = 0.03) than that for the AC patients who had pituitary dysfunction of only one axis. The composition of CR lesions was cystic (38%), solid (10%), or mixed solid and cystic (43%). Patients with RCC or AC groups had a significantly greater proportion (P = 0.006) of purely cystic lesions (88% and 100%, respectively). Calcification detectable on computed tomographic scanning was present in 87% of patients with CR, a significantly greater proportion (P < 0.001) compared to those with RCC (13%) or AC (0%). No significant differences were found between the groups based on computed tomography density, the presence of postcontrast enhancement, or magnetic resonance imaging. Recurrence rate was 62% for CR, 19% for RCC, and 20% for AC. Surgical intervention statistically improved most neurological, ophthalmological, and psychiatric manifestations; in contrast, galactorrhea, menstrual dysfunction, and diabetes insipidus (52% CR; 31% RCC) did not improve or became worse postoperatively. A significantly higher percentage of patients with CR required postoperative hormone replacement. Similarly, there was a biochemical trend suggesting that a smaller proportion of patients with CR improved in at least one pituitary axis after surgery (P = 0.08) compared to those with RCC or AC. There was a positive correlation between cyst size and recurrence rate (r = 0.689; P < 0.01) and between cyst size and time to recurrence (r = 0.582; P = 0.037) for all three groups. We describe the largest clinical, biochemical, radiographic, and histological series of adult patients with cystic disease of the sella turcica. Patients with AC tended to be older at initial diagnosis than CR or RCC patients. Mass effects, such as visual problems and headaches, are common symptoms of all three cystic lesions, but psychiatric deficits favor a diagnosis of CR. Calcification or solid components on neuroimaging characterize CR. Endocrinological deficits, especially diabetes insipidus, had the worst prognosis after surgery. Low recurrence rates can be expected for RCC and AC. These data have direct implications for the management and monitoring of patients with cystic lesions of the sella turcica.