Surgical management of craniopharyngiomas. A review of 74 cases.

A series of 74 patients with craniopharyngiomas were treated during a 15-year period. Of the 74 patients, 40 were males and 34 were females, with a mean age of 27 years (range 3 to 65 years). Twenty-eight patients (38%) were less than 18 years of age. Remission was defined as clinical improvement with stable ophthalmological and neurological status, radiological evidence of a decrease in tumor size, and either a continued decrease or a stable tumor size on follow-up radiological evaluations. A fair result was considered remission with new neurological deficits related to surgical intervention. All other results were considered a failure. The mean follow-up period in this study was 4 years, with 100% of the patients monitored. In children, the most common presentation was that of growth failure (93%). In adults, sexual dysfunction was the most common presentation, with 88% of males presenting with impotence or marked decrease in sexual drive, and 82% of females presenting with primary or secondary amenorrhea, often associated with galactorrhea. Considering the pediatric and adult populations together, the most common presenting symptom was visual dysfunction, with 71% of patients presenting in this manner. Fifty percent of patients presented with severe headache. The most frequent preoperative finding was a visual field defect, with 72% of patients so affected; 42% of patients had preoperative hypothyroidism and 24% had hypoadrenalism. Diabetes insipidus was present preoperatively in 23%. Hydrocephalus was uncommon, being present in only 15%. A subfrontal craniotomy was used in 47% of patients, a transsphenoidal approach in 39%, a subtemporal approach in 11%, a transcallosal approach in 5%, and a suboccipital craniectomy in 2%. Multiple procedures were required in 15% of patients in order to provide significant relief of compressive symptomatology. The results of therapy indicate that total tumor removal was deemed to have been achieved in only seven patients, six of whom have had no recurrence. However, 91% of patients are in remission, one had a fair result, and two died as a direct result of surgical intervention. One patient died from uncontrolled disease, and three patients died from unrelated causes. The results of this study indicate that radical subtotal removal followed by radiotherapy is an acceptable treatment for craniopharyngioma.