Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University, Rome

Craniopharyngioma (CP) is the most common intracranial non-glial tumour observed in pediatric age. Although histologically benign and amenable to surgical treatment, its location and relation with vital nervous and vascular structures makes the feasibility of a radical resection difficult even in the microneurosurgery era. Beside the difficulties experienced when performing tumour resection, post-operative complications, such as endocrinological imbalance, represent another point that makes CP total excision a challenge. In order to avoid such complications, some authors have suggested to renounce to radical resection and to rely on post-operative radiation therapy to minimise the risk of residual tumour progression.

We report our experience with 52 children and adolescents operated on for CP at the Department of Pediatrics, Section of Pediatric Neurosurgery, Catholic University Medical School, Rome, between January 1985 and December 2002. The study included 14 children <5 years old (five less than 2 years of age), 25 between 6 and 10 years old, and 13 more than 10 years old. The most common presenting signs were related to endocrinological imbalance (35 cases), increased intracranial pressure (32 cases), and to a lesser extent, visual compromise (17 cases). Concerning location, CP was intrasellar in three cases; sellar/suprasellar with prominent prechiasmatic growth in 24 cases; retrochiasmatic/3rd ventricular in 14 cases, and giant (with an extension into the middle and/or posterior cranial fossae) in 11 cases. The tumour was managed by means of a single surgical approach in 47 cases and with a two-stage operation in the remaining five cases. In 11 cases of intrasellar or intra/suprasellar midline location, the first surgical approach was done through the transsphenoidal route (which represented the first step of a staged operation in five instances); in the remaining 41 patients, craniotomy was the first surgical procedure. Radical tumour resection was achieved in 40 cases, subtotal (only small tumour remnants adherent to the carotid arteries, 3rd ventricle floor or visual pathways) in nine, and only partial in the remaining three cases

Histology demonstrated the adamantinous variant in all cases. Two surgical deaths were recorded in this series (both following a transsphenoidal approach): one secondary to uncontrollable intra-operative bleeding from the carotid artery, and the other to fulminating bacterial meningoencephalitis. Morbidity included endocrinological disturbances, namely hypopituitarism and diabetes insipidus, in more than 80% of cases, worsening of pre-operative visual deficit in six cases, and transitory neurological deficits in five cases. One late death was recorded 2 years after surgery, secondary to electrolytic imbalance although favoured by a major head trauma with subacute subdural haematoma. Nine recurrences occurred 1–8 years after surgery (three true recurrences, and six re-growths of incompletely resected tumours) that required re-operation. Twelve patients underwent radiotherapy, six after an initially incomplete tumour resection and six following relapse. One patient presented with a malignant thalamic glioma 8 years after radiotherapy. At long-term follow-up, all survivors showed good clinical condition, even though approximately 60% relied on hormone replacement, and some patients presented obesity. Diabetes insipidus has subsided in about 80% of the cases; visual deficits improved or remained stable, whereas post-operative neurological deficits subsided in all but one patient.

In our experience, radical resection of CP represented the first and almost unique treatment modality. Although not insignificant, post-operative mortality and morbidity do not seem to represent a major contraindication in attempting a radical tumour resection whenever possible. On the other hand, extensive hypothalamic involvement should suggest a less aggressive attitude.