Craneofaringiomas: riesgos y desafíos del abordaje endonasal endoscópico extendido a la base del cráneo / Craniopharyngiomas: risks and challenges of extended endoscopic endonasal approach to the skull base

Introducción: Los craneofaringiomas, son tumores frecuentes y aunque son histológicamente benignos, plantean importantes problemas terapéuticos por su naturaleza y morbilidad asociada a la cirugía, siendo una lesión desafiante para los neurocirujanos. Material y Método: Se realizó un estudio descriptivo de los resultados alcanzados con el abordaje endonasal endoscópico extendido trans-tubérculo trans-plano en una serie de pacientes con diagnóstico de craneofaringioma en el período de 2009 al 2012 en el Hospital Clínico Quirúrgico “Hermanos Ameijeiras”. Resultados: Se intervinieron 37 pacientes, (23 F/14 M). Se logró la resección total macroscópica en 34 pacientes. La complicación más frecuente fue la diabetes insípida presentada en 16 enfermos. Tuvimos 4 fallecidos: 3 de ellos por complicaciones médicas en el postoperatorio tardío. Conclusiones: El abordaje endonasal endoscópico extendido trans-tubérculo trans-plano permite acometer el tratamiento quirúrgico favoreciendo una resección amplia para este tipo de lesión. Con tecnología aun en desarrollo este abordaje representa una alternativa esperanzadora para los pacientes con craneofaringiomas.

Introduction: The craneopharyngiomas, are frequent tumors and although they are histological benign lesion, they outline therapeutic important problems for their nature and morbidity associated to the surgery, being a defiant lesion for neurosurgeons. Material and Methods: In this article, we present the results using Extended Endonasal Endoscopic Approach trans-tuberculum trans-planum in patient with craniopharyngioma in “Hermanos Ameijeiras” Hospital between 2009 and 2012. Results: 37 patients were operated, (23 F/ 14 M). We achieve gross total resection in 34 patients. The most frequent complication was insipid diabetes presented in 16 patients. We had 4 deaths, 3 of them were for postoperative medical complication. Conclusions: The extended endonasal endoscopic approach trans-tuberculum trans-planum allows performing the surgical treatment with a wide resection in this type lesion. With technology even in development this approaches represents an alternative for patients with craniopharyngioma.

Treatment of craniopharyngioma in adults: systematic analysis of a 25-year experience.

BACKGROUND AND AIMS: Craniopharyngioma is a rare and mostly benign epithelial tumor of the central nervous system, mostly affecting children. Considering that most of the published series of craniopharyngioma are based on pediatric populations, studies in adults gain importance based mainly on the reduced number of cases and the possible differences emerging from a mostly different histological type. We undertook this study to establish the pattern of presentation, morphological features and specific characteristics of craniopharyngioma in an adult Mexican population, as well as discussing the long-term outcome and how it may be influenced by surgical, anatomic and clinical factors. METHODS: A total of 153 adult patients (16 years or older) underwent transcranial and transsphenoidal surgery between January 1985 and December 2009, all with histological confirmation of craniopharyngioma. Hypothalamic involvement, surgical complications, rate of tumor resection and endocrinological outcome were evaluated. RESULTS: Seventy nine males (51.6%) and 74 females (48.4%) were included. Mean age at diagnosis was 32.4 years (range: 16-77 years). Mean initial tumor volume was 28.44 mL (range: 0.18-100.44 mL). Partial or complete hypothalamic involvement (Samii Grades III, IV and V) was found in 90.2%. The overall rate of new endocrinopathies was 37.25% after surgery (95% CI = 33.9-41.2). CONCLUSIONS: Gross total removal of craniopharyngiomas with large hypothalamic involvement was related to poor neuroendocrine outcome in adults. Partial removal should be indicated, associated with adjuvant therapy, in order to improve postoperative neuroendocrine status.

Complications in paediatric craniopharyngioma treatment.

Forty-eight consecutive children treated for craniopharyngioma at the Juan P. Garrahan National Paediatric Hospital (Buenos Aires, Argentina) from 1988 to 1994 are described. Complications of patients undergoing total resection alone and those undergoing subtotal or partial resection plus radiotherapy were compared. Survival time and quality of life proved more satisfactory in the former group, as there were no recurrences. In contrast, among the latter patients, 53% suffered relapses. Endocrinological complications were similar in the two groups. Postsurgical subdural haematomas were quite frequent and eight patients required treatment for intracranial hypertension. Vascular complications, though less common, led to high morbidity and mortality. There was a considerable incidence of shunt malfunction (80%), arguing against placement of a preoperative shunt, which tended besides to foster postsurgical subdural haematomas.

Pediatric craniopharyngiomas: long term results of combined treatment with surgery and radiation.

From 1961 to 1981, 19 pediatric patients with craniopharyngiomas were treated with external beam radiation post surgery. Twelve underwent primary treatment while seven were treated for recurrence. Median follow-up is 21 years (range 8-28 years) with an overall 20-year survival of 62%. Twenty-six percent (5/19) developed disease recurrence following radiation. Of the multiple patient and treatment parameters analyzed, only initial disease status (i.e., primary vs recurrence), surgical extent, radiation dose, and treatment prior to routine use of CT scans (1961-1974) appeared to have major influence on patient outcome. The 20-year survival for those treated for primary disease was 78% versus 25% for those treated for recurrence. Fifty percent (3/6) of patients receiving tumor doses of < or = 5400 cGy developed recurrences versus 15% (2/13) in those receiving > 5400 cGy. Four of the five recurrences occurred in patients treated during the pre-CT era. Long term effects of treatment were analyzed. Surgical morbidity correlated strongly with extent of surgery. All patients who underwent total resection developed surgical sequelae. Radiation morbidity correlated strongly with radiation dose. The incidence of vascular or neurologic complications attributable to radiation were minimal, occurring in only two patients. Both received doses higher than that given with modern therapy. Long-term results from this series are better than those reported with surgery alone. Use of modern surgical and radiation equipment/technique along with CT/MRI imaging should yield improved treatment results both in terms of lower recurrence and lower toxicity rates.