Faculty Bibliography

OBJECT: Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise. METHODS: The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction). RESULTS: There was a significant increase in pituitary dysfunction following treatment-consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas-and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus. CONCLUSIONS: Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors

OBJECT: Fusiform dilation of the supraclinoid internal carotid artery (FDCA) is a reported occurrence following surgery for suprasellar tumors, in particular craniopharyngiomas. We report our experience of the incidence and natural history of FDCA following aggressive surgical resection of craniopharyngiomas in children. METHODS: Between 1986 and 2006, 86 patients under the age of 21 underwent radical resection of craniopharyngiomas at our institution. Ten cases with < 1 year of follow-up imaging (6), perioperative death (3), or nonsuprasellar tumors (1) were excluded. Data were retrospectively collected on the remaining 76 patients (43 male, 33 female; mean age 9.5 years; mean tumor size 3.3 cm) to determine the risk factors for and the rate and clinical significance of FDCA. RESULTS: Fifty patients had primary tumors and 26 patients received treatment before referral to our center. Sixty-six children (87%) had gross-total resection. At a mean follow-up time of 9.9 years, FDCA had developed in 7 patients (9.2%), all of whom had primary tumors and gross-total resection. The mean time to onset of FDCA was 6.8 months (range 3-11 months) with stabilization occurring at mean of 17.7 months (range 9-29.5 months). The mean size of the aneurysms was 9.1 mm (range 7.1-12 mm). After arrest, no lesions showed continued growth on serial imaging or produced symptoms or required treatment. There were no significant differences in age, sex, tumor size, pre- or retrochiasmatic location, extent of resection, or surgical approach (p > 0.05) between patients with and without FDCA. CONCLUSIONS: Fusiform dilation of the supraclinoid internal carotid artery occurred in almost 10% of children following radical resection of craniopharyngiomas. In agreement with other reports, the authors concluded that FDCA probably occurs as a result of surgical manipulation of the supraclinoid carotid artery and should be managed conservatively because very few patients exhibit continued symptoms or experience growth or rupture of the lesion

OBJECT: Optimal treatment of primary and recurrent craniopharyngiomas remains controversial. Radical resection and limited resection plus radiation therapy yield similar rates of disease control and overall survival. The data are much less clear for recurrent tumors. The authors report their experience with radical resection of both primary and recurrent craniopharyngiomas in children and compare the outcomes between the 2 groups. METHODS: A retrospective analysis was performed in 86 children younger than 21 years of age who underwent a total of 103 operations for craniopharyngioma between 1986 and 2008; these were performed by the senior author. The goal was resection with curative intent in all patients. Two patients were lost to follow-up and were excluded from analysis. The mean age at the time of surgery was 9.6 years, and the mean follow-up was 9.0 years. RESULTS: All 57 children with primary tumors underwent gross-total resection (GTR). A GTR was achieved in significantly fewer children with recurrent tumors (18 [62%] of 29). There were 3 perioperative deaths (3%). Tumor recurred after GTR in 14 (20%) of 71 patients. Overall survival and progression-free survival were significantly better in patients with primary tumors at time of presentation to the authors' institution. There were no significant differences in the neurological, endocrinological, visual, or functional outcomes between patients with primary and those with recurrent tumors. Factors negatively affecting overall survival and progression-free survival include subtotal resection (recurrent tumors only), tumor size >or= 5 cm, or presence of hydrocephalus or a ventriculoperitoneal shunt. Prior radiation therapy and increasing tumor size were both risk factors for incomplete resection at reoperation. CONCLUSIONS: In the hands of surgeons with experience with craniopharyngiomas, the authors believe that radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity. While GTR does not preclude recurrence and is more difficult to achieve in recurrent tumors, especially large and previously irradiated tumors, radical resection is still possible in patients with recurrent craniopharyngiomas with morbidity similar to that of primary tumors

We present a case of synchronous involvement of the pineal and suprasellar regions by a mixed germ cell tumor comprising germinoma and yolk sac tumor components, with a predominant angiomatous component. To our knowledge, it is the first case of this nature to be reported in the literature. Usually, synchronous lesions of this kind are pure germinomas, and some clinicians will forgo a biopsy and assume a germinoma histology if the serum beta-human chorionic gonadotrophin (HCG) is <50 IU/l and the alpha-fetoprotein (AFP) is within normal limits. Secondly, if a biopsy is performed on a lesion that has a prominent angiomatous component, the diagnostic germ cell tumor may be missed at the time of the biopsy. In order to alert clinicians and pathologists to this rare entity, the case is discussed with particular reference to difficulties that were encountered in rendering an accurate diagnosis, and the associated management implications

We present our technique for extraoperative functional mapping in awake children with supratentorial tumors near eloquent cortex. This technique may help optimize tumor resection and/or the functional outcome, especially when an awake craniotomy is not possible or in the setting of coincident seizures. After subdural electrode implantation, extraoperative functional mapping and seizure focus mapping were performed, followed by staged tumor resection. Gross total resection was achieved in 6 patients and subtotal resection in 2. No complications due to electrode placement or invasive monitoring were observed. Staged resection of pediatric brain tumors near the eloquent cortex appears safe, allows functional mapping in awake patients, and may assist in decisions between maximal tumor resection and optimal functional outcome

OBJECT: Given the potential morbidity of cranial irradiation in young children, the risk-benefit analysis of limited surgery plus irradiation versus radical resection may favor the latter strategy. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes of patients 5 years of age and younger who underwent radical resection of craniopharyngiomas. METHODS: Between 1991 and 2008, 19 children age < or = 5 years were diagnosed with a craniopharyngioma and underwent radical resection by the senior author (J.H.W.). Data were retrospectively collected on these 19 patients (11 males, 8 females; mean age 3.2 years) to assess the efficacy and impact of surgical treatment. RESULTS: Eighteen (95%) of 19 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. There was no operative death and 18 of (95%) 19 patients were alive at a mean follow-up of 9.4 years (median 8.3 years). Six patients (31%) had a total of 7 tumor recurrences treated by repeat GTR in 5 patients and Gamma knife surgery in 1 patient. No patient required conventional, fractionated radiation therapy. Disease control was achieved surgically in 17 (89.5%) patients and with surgery and Gamma knife surgery in 1 patient, yielding an overall rate of disease control of 95% without the use of conventional radiotherapy. New-onset diabetes insipidus occurred in 50% of patients. Vision worsened in 1 patient, and there was no long-term neurological morbidity. CONCLUSIONS: In this retrospective series, children aged < or = 5 years with craniopharyngiomas can have excellent outcomes with minimal morbidity after radical resection by an experienced surgeon. Disease control in this population can be successfully achieved with GTR alone in the majority of cases, avoiding the detrimental effects of radiotherapy in this vulnerable population