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Vol. 34. Issue 2.
Pages 67-74 (March - April 2023)
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Vol. 34. Issue 2.
Pages 67-74 (March - April 2023)
Clinical Research
Multicentric and collaborative study of Spanish neurosurgical management of pediatric craniopharyngiomas: S-PedCPG.co
Estudio multicéntrico colaborativo del manejo neuroquirúrgico del craneofaringioma pediátrico en España: S-PedCPG.co
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Sara Iglesiasa,
Corresponding author
siglesias95@yahoo.es

Corresponding author.
, Pablo M. Munarrizb, Javier Sacedac, Gregorio Catalán-Uribarrenad, Pablo Mirandae, Juana M. Vidalf, David Fusterog, Jorge Giménez-Pandoh, Francisca Riusi
a Neurosurgery Department, Hospital Regional Universitario de Málaga, Málaga, Spain
b Neurosurgery Department, Hospital Universitario 12 de Octubre, Madrid, Spain
c Neurosurgery Department, Hospital Universitario La Paz, Madrid, Spain
d Neurosurgery Department, Hospital Universitario Cruces, Bilbao, Spain
e Neurosurgery Department, Hospital Universitario y Politécnico La Fe, Valencia, Spain
f Neurosurgery Department, Hospital Universitario Reina Sofía, Córdoba, Spain
g Neurosurgery Department, Hospital Universitario Miguel Servet, Zaragoza, Spain
h Neurosurgery Department, Hospital Universitario de Badajoz, Badajoz, Spain
i Statistics Department, Universidad de Málaga, Spain
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Abstract
Purpose

To present a descriptive analysis of pediatric craniopharyngiomas (PedCPG) treated in various Spanish hospitals, defining factors related to recurrence and performing a critical analysis of the results.

Methods

We undertook a multicenter retrospective review of PedCPG treated between 2000 and 2017. Data collected included epidemiological variables, clinical and radiological characteristics, goal of first surgery, rate of recurrence and its approach, adjuvant treatment, complications and permanent morbidity. Associations were studied between progression and number of progressions and independent variables.

Results

The study involved 69 children from 8 Spanish hospitals. Most of the tumors invaded several intracranial compartments at diagnosis, with the hypothalamus involved in 41.3% of cases. The first treatment strategy was usually gross total resection (GTR) (71%), with some patients treated with radiotherapy or intracystic chemotherapy. The progression rate after first surgery was 53% in a mean follow-up of 88.2 months (range 7–357). In the GTR group 38.8% of tumors recurred, 40% in the group of subtotal resection or biopsy and 93.3% in the cyst fenestration±Ommaya reservoir group. Mortality was 7.2%. Follow-up period, size of the tumor and goal of first surgery were significantly related with progression.

Conclusions

Our results in terms of disease control, hormonal or visual impairment and mortality were acceptable, but there are several areas for improvement. Our short-term goals should be to create a national register of PedCPG, reach a consensus about a treatment algorithm, and improve diagnosis of hypothalamic dysfunction to avoid preventable morbidity.

Keywords:
Pediatric craniopharyngioma
Multimodal treatment
Progression
Local control
Quality of life
Algorithms
Resumen
Objetivo

Presentar un análisis descriptivo de los craneofaringiomas pediátricos tratados en varios hospitales españoles, definiendo los factores relacionados con la recurrencia y realizando un análisis crítico de los resultados.

Métodos

Estudio retrospectivo multicéntrico de los craneofaringiomas pediátricos tratados entre 2000-2017. Se recogieron variables epidemiológicas, clínicas y radiológicas, el objetivo de la primera cirugía, la tasa de recurrencia y su abordaje, los tratamientos adyuvantes, así como las complicaciones y la morbilidad permanente. Se estudió la relación estadística entre la progresión y el número de progresiones con las variables independientes.

Resultados

Se incluyeron 69 niños tratados en 8 hospitales españoles. La mayoría de los tumores se extendían por varios compartimentos intracraneales al diagnóstico, con invasión hipotalámica en el 41,3%. Habitualmente, la primera estrategia de tratamiento fue la resección radical (71%), con algunos pacientes tratados con radioterapia o quimioterapia intraquística. La tasa de progresión tras la primera cirugía fue del 53% en un seguimiento medio de 88,2 meses (rango 7-357). En el grupo de resección radical recurrieron un 38,8% de los tumores, un 40% en el de resección subtotal o biopsia y un 93,3% en el de fenestración quística±reservorio Ommaya. La mortalidad fue de un 7,2%. Las variables relacionadas de forma significativa con progresión fueron el tiempo de seguimiento, el tamaño del tumor y el objetivo de la primera cirugía.

Conclusiones

Los resultados obtenidos fueron aceptables en control de la enfermedad, secuelas hormonales o visuales y mortalidad, aunque hay varias áreas susceptibles de mejora. Nuestros objetivos a corto plazo deberían estar orientados a crear un registro nacional de craneofaringiomas pediátricos, alcanzar un consenso respecto al algoritmo de tratamiento y mejorar el diagnóstico de la disfunción hipotalámica para evitar morbilidad.

Palabras clave:
Craneofaringioma pediátrico
Tratamiento multimodal
Progresión
Control local
Calidad de vida
Algoritmos

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