Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?

Abstract

Seventy-five children treated for craniopharyngioma between 1973 and 1994 were studied to demonstrate which pre- and intraoperative factors were indicative of a poor outcome as defined by a quantitative assessment of morbidity. This involved a retrospective review of 65 patients and a prospective study of 10 patients focused on clinical details and cranial imaging and a follow-up "study assessment" of 66 survivors performed over the last 2 years. As part of the assessment, 63 patients underwent magnetic resonance imaging with a three-dimensional volume acquisition sequence 1.5 to 19.2 years after initial surgery. Predictors of high morbidity included severe hydrocephalus, intraoperative adverse events, and young age ( < or = 5 years) at presentation. Predictors of increased hypothalamic morbidity included symptoms of hypothalamic disturbance already established at diagnosis, greater height ( > or = 3.5 cm) of the tumor in the midline, and attempts to remove adherent tumor from the region of the hypothalamus at operation. Large tumor size, young age, and severe hydrocephalus were predictors of tumor recurrence, whereas complete tumor resection (as determined by postoperative neuroimaging) and radiotherapy given electively after subtotal excision were less likely to be associated with recurrent disease. Based on these findings, the authors propose an individualized, more flexible treatment approach whereby surgical strategies may be modified to provide long-term tumor control with the lowest morbidity.

Angiogenesis in patients with craniopharyngiomas

“…Advances have been made in identifying subsets of patients who are at particularly greater risk of developing recurrent tumors. 26,27 Predictors of poor prognosis include large tumor size, young age, severe hydrocephalus, and hypopituitarism. Conversely, macroscopic total resection as well as subtotal excision followed by radiotherapy were less likely to be associated with tumor recurrence.…”

“…Conversely, macroscopic total resection as well as subtotal excision followed by radiotherapy were less likely to be associated with tumor recurrence. 9,10,[27][28][29][30] Despite therapeutic advances, craniopharyngiomas often recur and may prove fatal. Therefore, it is important to find an accurate method of identifying patients who are at risk for disease progression.…”

Spontaneous Resolution of Diabetes Insipidus After Pituitary Stalk Sectioning During Surgery for Large Craniopharyngioma-Endocrinological Evaluation and Clinical Implications for Surgical Strategy-

“…However, the surgical outcomes for large craniopharyngiomas remain controversial. 1,6,10,11,[15][16][17]19,[22][23][24][27][28][29]31,[34][35][36][37][38][39][40][41][42]44,45) Furthermore, most reports on craniopharyngioma have focused primarily on children 10,[15][16][17]22,27,41,42) or mixed series, 1,19,23,24,31,34,36,[38][39][40]45) and studies of only adults are limited. 6) The postoperative endocrinological state of patients treated for craniopharyngioma is critical to the quality of life.…”

‘Do no harm’: management of craniopharyngioma

“…Ninety per cent of patients develop DI post-operatively which will remain permanent in 60-80% (8,14). The diagnosis of DI is relatively straightforward and management involves a combination of judicial fluid balance and DDAVP administration.…”

“…Complete excision is likely when tumours are less than 2 cm in diameter, but not so if greater than 4 cm (12,13). Nearly half of craniopharyngiomas are greater than 3 cm in diameter at diagnosis (12,14). In patients with larger tumours, who have hydrocephalus or hypothalamic involvement and undergo radical resection, there is a high likelihood of hypothalamic morbidity (O50%), operative mortality (2.5-10%) and late mortality (11-28%) (15,16).…”