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Phiroz E. Tarapore, Michael E. Sughrue, Lewis Blevins, Kurtis I. Auguste, Nalin Gupta, and Sandeep Kunwar

Object

Pituitary adenomas are uncommon in childhood. Although medical treatment can be effective in treating prolactinomas and some growth hormone (GH)–secreting tumors, resection is indicated when visual function is affected or the side effects of medical therapy are intolerable. The authors of this report describe their 10-year experience in managing pituitary adenomas via the microscopic endonasal transsphenoidal approach in a pediatric population.

Methods

They performed a retrospective review of a surgical case series based at a single institution and consisting of 34 consecutive pediatric patients with endocrine-active (32 patients) and endocrine-inactive (2 patients) adenomas. These patients were surgically treated via an endonasal transsphenoidal approach between 1999 and 2008. Patient charts were reviewed, and clinical data were compiled and analyzed using the chi-square and Kaplan-Meier tests.

Results

The patient cohort consisted of 20 girls and 14 boys, with ages ranging from 9 to 18 years and a median age of 16 years. Thirty-two patients (94%) underwent surgery for endocrine-active tumors, including 10 (29%) with Cushing disease, 21 (62%) with prolactinomas, and 1 (3%) with GH-secreting tumors. Two patients with nonsecreting adenomas underwent surgery for apoplexy. The mean tumor volume was 5.4 cm3, and 13 patients (38%) had suprasellar extension and 7 (21%) had cavernous sinus invasion. Gross-total resection was achieved in 26 patients (76%), although it was significantly less likely to be achieved in the setting of cavernous sinus invasion (p < 0.001) but was unaffected by suprasellar extension. Residual tumor was treated with radiation therapy in 6 patients (18%). The average duration of hospital stay was 1.6 days. The median follow-up time was 18 months. After surgery, 19 patients (56%) had normal hormone function without adjuvant therapy, 8 (24%) had normal function with adjuvant therapy, and 5 (15%) had persistently elevated hormone levels. Patients with a macroprolactinoma were significantly more likely to require postoperative adjuvant therapy than were those with a microprolactinoma (p < 0.03).

Conclusions

Endonasal transsphenoidal resection is a safe, well-tolerated, and potentially curative treatment option for pituitary adenomas in children. Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity. Endocrine function is usually preserved, and the majority of patients will not require lifelong medical therapy.

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Arman Jahangiri, Annette M. Molinaro, Phiroz E. Tarapore, Lewis Blevins Jr., Kurtis I. Auguste, Nalin Gupta, Sandeep Kunwar, and Manish K. Aghi

Object

Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC.

Methods

The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008.

Results

In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5).

Conclusions

The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.