Multicentric and collaborative study of Spanish neurosurgical management of pediatric craniopharyngiomas: S-PedCPGco / Estudio multicéntrico colaborativo del manejo neuroquirúrgico del craneofaringioma pediátrico en España: S-PedCPGco

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 (AU)

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 (AU)

Multicentric and collaborative study of Spanish neurosurgical management of pediatric craniopharyngiomas: S-PedCPG.co.

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.

Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature / Recurrencia ectópica de craneofaringioma en fosa posterior: presentación de casos y revisión de la literatura

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection + radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved. (AU)

Los craneofaringiomas son tumores epiteliales benignos que pueden recurrir ocasionalmente en localizaciones ectópicas. Presentamos 2 casos de recurrencias ectópicas, ambos en fosa posterior, y realizamos una revisión de la literatura con análisis estadístico básico. Dos pacientes ingresaron en nuestro servicio con lesiones en fosa posterior en quienes se indicó resección quirúrgica. El diagnóstico histopatológico fue de craneofaringioma adamantinomatoso en ambos casos. Ambos pacientes fueron intervenidos años atrás por un tumor supraselar. Se hallaron 67 casos descritos de recurrencia ectópica de craneofaringioma (incluyendo el presente estudio): 51 fueron adamantinomatoso (76%), 6 papilar (9%) y 10 desconocidos (15%). Dieciocho casos ocurrieron en la fosa posterior, todos con diagnóstico de craneofaringioma adamantinomatoso. El intervalo hasta la recurrencia fue de 15,15 años para aquellas en la fosa posterior y de 5,75 años para recurrencias supratentoriales. El test de Student mostró diferencias estadísticamente significativas en el tiempo de recurrencia (p 0,002). En 53 casos se obtuvo una resección completa (79%), resección subtotal + radioterapia en 3 (5%) y 11 casos se usaron otras modalidades de tratamiento (16%). La recurrencia ectópica es un evento raro, pero posible. Aquellas en la fosa posterior pueden aparecer más tarde que a nivel supratentorial. Los craneofaringiomas adamantinomatosos son los subtipos más probables en esos casos, posiblemente debido a su comportamiento más agresivo comparado con los papilares. Un seguimiento a largo plazo debe llevarse a cabo para detectar dichas recurrencias ectópicas, ya que estas suelen ser quirúrgicamente accesibles, y debe obtenerse una resección completa. (AU)

Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature.

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection+radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved.

Características clínicas e histopatológicas de niños peruanos con craneofaringioma

Introducción: Los craneofaringiomas son tumores benignos, de los cuales hasta el 50% ocurren en niños. Sin embargo, no hay estudios en niños peruanos. Objetivo: describir las características clínicas e histopatológicas de niños con craneofaringioma hospitalizados en el Hospital Nacional Edgardo Rebagliati Martins entre agosto de 2019 y mayo de 2021. El estudio: El diseño es transversal y la fuente de los datos fueron las historias clínicas. Se recolectó información sobre el sexo, edad, cirugías y características relacionadas a la primera cirugía de resección tumoral. Hallazgos: Incluimos 12 pacientes. El 83.3% fueron varones, al momento del diagnóstico la mediana de edad fue de 6 años y predominaron los síntomas visuales, todos tuvieron deficiencias hormonales luego de la primera cirugía de resección. Conclusión: tres recibieron radioterapia, uno desarrolló transformación maligna y otro falleció. Es necesario realizar a futuro estudios prospectivos.

Introduction: Craniopharyngiomas are benign tumors, of which up to 50% occur in children. However, there are no studies in Peruvian children. Objective : to describe the clinical and histopathological characteristics of children with craniopharyngioma hospitalized at the Edgardo Rebagliati Martins National Hospital between August 2019 and May 2021. The study: The design is cross-sectional and the data source were medical records. Information on sex, age, surgeries and characteristics related to the first tumor resection surgery were collected. Findings: We included 12 patients. 83.3% were male, at the time of diagnosis the median age was 6 years and visual symptoms predominated, all had hormonal deficiencies after the first resection surgery. Conclusions: three received radiotherapy, one developed malignant transformation and one died. Future prospective studies are necessary.

A rare case of a secondary pituitary abscess arising in a craniopharyngioma with atypical presentation and clinical course / Un caso raro de absceso hipofisario secundario derivado de un craneofaringioma con presentación y evolución clínica atípicas

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study demonstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely (AU)

Los abscesos hipofisarios son infrecuentes. Se pueden dividir en primarios o secundarios, si se producen sobre una lesión previa. Presentamos el octavo caso de un absceso asentado sobre un craneofaringioma. Una mujer de 59 años consultó por fiebre y cefalea de tres semanas de evolución. La exploración física era anodina. Una resonancia magnética (RMN) evidenció una lesión hipofisaria sugestiva de un proceso inflamatorio crónico. Finalmente, se diagnosticó de una meningitis linfocítica e hipofisitis y se trató con corticoides. Dos meses después reconsultó por los mismos síntomas. En la RMN se evidenció crecimiento de la lesión, por lo que se biopsia endoscópicamente. Durante el procedimiento salió pus. En el examen histológico se evidenció un craneofaringioma y una inflamación meníngea. Se iniciaron antibióticos empíricamente. En el seguimiento a tres meses, la RMN evidenciaba un dudoso resto. Los abscesos hipofisarios secundarios son raros y hay que tener un alto índice de sospecha para diagnosticarlos. El tratamiento se basa en antibioterapia y evacuación transesfenoidal. Aunque los síntomas se suelen resolver, las endocrinopatías no (AU)

A rare case of a secondary pituitary abscess arising in a craniopharyngioma with atypical presentation and clinical course.

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study demonstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely.

Resultados clínicos y quirúrgicos del tratamiento de craneofaringiomas en pediatría / Clinical and surgical results of the treatment of craniopharyngiomas in pediatrics

Introducción: Los craneofaringiomas son tumores benignos frecuentes en pediatría. La cirugía resectiva solía ser el tratamiento de elección. Sin embargo, en los últimos años se encuentra muy cuestionada debido a su elevada morbilidad. El objetivo primario de este trabajo es presentar los resultados clínicos y quirúrgicos de la cirugía resectiva de craneofaringiomas por vía transcraneal en nuestra institución. El objetivo secundario es presentar una serie de casos en los que se realizó un tratamiento quirúrgico conservador utilizando un reservorio de Ommaya para tratamiento del componente quístico. Métodos: Estudio descriptivo de una serie de casos que compara evaluaciones clínicas e imagenológicas pre y postoperatoria en pacientes sometidos a cirugía resectiva de craneofaringioma. Además, se presenta una serie de casos de pacientes con craneofaringiomas predominantemente quísticos tratados con colocación de catéter de Ommaya intraquístico e Interferón. Resultados: Se analizaron 33 pacientes con craneofaringioma sometidos a resección quirúrgica. Al año de la cirugía, encontramos que el 51.52% de los casos (17 pacientes) se mantuvo sin crecimiento de la lesión y que el 48.48% (16 pacientes) presentó aumento o recidiva. En el postoperatorio todas las evaluaciones clínicas empeoraron. Fueron estadísticamente significativas la aparición de diabetes insípida (18.18% vs. 69.70%; p=0.04), obesidad (12.12% vs. 36.36%; p=0.005) y talla baja (33.33% vs. 57.58%; p=0.0006). En los tratados con Ommaya e Interferón, el 28.57% (2 casos) presentó reducción o estabilidad en el quiste y el 71.43% (5 casos) aumentó el tamaño, aunque presentó mejores resultados respecto a la restricción de la talla y obesidad. Conclusión: La exéresis quirúrgica de los craneofaringiomas genera una alta tasa de comorbilidades sumado a un porcentaje elevado de recidiva. El manejo conservador tratando el componente quístico en los casos en los que sea posible podría ser una opción viable. El tratamiento de los craneofaringiomas de la infancia debe abordarse de forma multidisciplinaria intentando preservar el bienestar y la calidad de vida de los pacientes

Introduction: Craniopharyngioma is a common benign tumor in children. Although surgery has traditionally been the treatment of choice, it has been controversial in recent years due to the associated high morbidity. The main aim of this paper is to present the clinical and surgical outcomes seen after craniopharyngioma resection in our institution. The secondary objective is to present a case series of patients treated with an Ommaya reservoir (OR) and interferon. Methods: This was a descriptive study comparing the pre and post-surgical clinical and image assessment in patients undergoing craniopharyngioma resection. Also, a case series including patients with craniopharyngioma managed with an Ommaya reservoir (OR) and Interpheron was included. Results: Thirty-three patients with craniopharyngioma undergoing surgical resection were assessed. We found no tumor growth in 51.52% (17) of the cases, and either tumor growth or recurrence in 48.48% of the cases at one year. Clinical worsening was observed in the post-operative period; the presence of diabetes insipidus (18.18% vs. 69.70%; p=0.04), obesity (12.12% vs. 36.36%; p=0.005) and short stature (33.33% vs. 57.58%; p=0.0006) were statistically significant. Of the patients treated with an OR and interferon, 28.57% (2) presented tumor cyst regression or stability, and 71.43% exhibited tumor cyst growth. Conclusion: The surgical resection of craniopharyngiomas is associated with a high recurrence rate and usually high mortality. A multidisciplinary management of craniopharyngiomas in childhood is advisable in order to preserve the wellbeing and quality of life of patients

A rare case of a secondary pituitary abscess arising in a craniopharyngioma with atypical presentation and clinical course.

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study demonstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely.

Craneofaringiomas: cirugía transnasal via endoscópica / Craniopharyngiomas: endoscopic transnasal surgery

Introducción: Debido a su ubicación anatómica, los craneofaringiomas son tumores complejos en su tratamiento. Una resección completa tiene riesgos elevados de morbimortalidad y si se opta por una resección parcial la tasa de recurrencia es alta. Según su extensión supraselar y su relación con el tallo hipofisario, pueden dividirse en preinfundibulares, transinfundibulares y retroinfundibulares. Objetivo: El objetivo de este video es describir la técnica vía endoscópica para la resección de un craneofaringioma preinfundibular. Materiales y Métodos: Se seleccionó un caso de un paciente con un craneofaringioma preinfundibular operado en el Servicio de Neurocirugía del Hospital Italiano de Buenos Aires, por vía endoscópica transnasal. Resultados: Se realizó una exéresis completa de la masa tumoral, sin evidenciar complicaciones tales como, diabetes insípida o fistula de líquido cefalorraquídeo y con recuperación completa del déficit campimétrico. Conclusión: El tratamiento quirúrgico de los craneofaringiomas requiere un conocimiento detallado de la anatomía de base de cráneo así como de las diferentes técnicas quirurgicas. El uso de la endoscopia ha permitido un mejor acceso a éste tipo de lesiones, disminuyendo las comorbilidades en el paciente y la estadía hospitalaria. Un resultado quirúrgico satisfactorio se obtiene con la resección completa y la menor morbilidad posible para el paciente

Introduction: Due to its anatomical location, craniopharyngiomas are difficult tumors to treat. Complete resection has high morbidity and mortality and if a partial resection is chosen, the recurrence is common. According to their suprasellar extension and its relationship with stalk, it can be classified into: preinfundibular, transinfundibular and retroinfundibular. Objetive: The aim of this video is to describe the surgical technique we use for preinfundibular craniopharyngioma. Methods: We review a preinfundibular craniopharyngioma operated on the Neurosurgery Department of the Hospital Italiano de Buenos Aires, through a transnasal endoscopic approach. Results: Total removal was achived, no acute complications were found such as diabetes insipidus or cerebrospinal fluid leak and improved their visual field. Conclusion: Craniopharyngioma surgery requires detailed knowledge of skull base anatomy and approaches. The use of endoscopy has allowed better access to this type of lesions, reducing comorbidities and patient hospital stay. Complete resection with low morbidity are the treatment of this tumors

Craneofaringioma: resección paso a paso en un paciente pediátrico / Craniopharyngioma: Step-by-Step Resection in a Pediatric Patient

Introducción: Describir detalladamente paso a paso la resección de un craneofaringioma por vía endoscópica endonasal en un paciente pediátrico, con el fin de guiar a neurocirujanos en formación. Materiales y métodos: Se describe detalladamente la técnica endoscópica endonasal transesfenoidal para la resección de un tumor de estirpe craneofaringioma adamantinomatoso en un paciente masculino de 3 años de edad con la variante de seno esfenoidal tipo conchal. Resultados: Se logró resección total de un craneofaringioma en paciente pediátrico mediante abordaje endoscópico endonasal. Se respetaron las estructuras vasculares circundantes y se pudo prevenir la fístula de líquido cefalorraquídeo con la utilización del flap nasoseptal. Conclusión: El abordaje endoscópico endonasal transesfenoidal ofrece una exposición amplia de la región selar y supraselar permitiendo una excelente resección de los craneofaringiomas en pacientes pediátricos. Consideramos de importancia la curva de aprendizaje para lograr una resección máxima sin agregar comorbilidades al paciente.

Objective: To give a detailed description, step by step, of endoscopic endonasal resection for craniopharyngioma in a pediatric patient. This manuscript was made to teach neurosurgeons in their former years. Methods: Detailed description with intraoperative images from a complete resection of adamantinomatous craniopharyngioma in a 3 years-old patient with a conchal variant sphenoid sinus. Results: We achieved a gross total resection of a craniopharyngioma in a pediatric patient by endoscopic endonasal approach. We preserve the surrounding vascular structures with no comorbidities. Cerebrospinal leak was prevented by using the nasoseptal flap covering the sphenoid defect. Conclusion: The endoscopic endonasal approach offers a wide exposure of the selar and supraselar región. This allowed us a great possibility for complete resection in selar and supraselar craniopharyngiomas in pediatric population. We consider that maximal resection should be balanced with a good learning curve to avoid comorbidities.

Cirugía endoscópica avanzada de base de cráneo, cavidad nasal y senos paranasales: experiencia de 40 meses en un hospital universitario / Advanced endoscopic surgery of skull base, nasal cavity and paranasal sinus: experience of 40 months in a university hospital / Cirugia endoscopica avançada de base de craneo, cavidade nasal e seios paranasales: experiência de 40 meses num hospital universitário

INTRODUCCIÓN: El uso del endoscopio junto con el avance de la tecnología ha facilitado la extensión de los límites tradicionales en la cirugía de la base del cráneo, la cavidad nasal y los senos paranasales. OBJETIVO: Describir los hallazgos epidemiológicos, clínicos e histopatológicos en los pacientes intervenidos por cirugía endoscópica avanzada en una unidad de Otorrinolaringología en 40 meses. MATERIAL Y MÉTODOS: Estudio retrospectivo. Los protocolos quirúrgicos e historias clínicas fueron revisados. Se realizó un análisis univariado descriptivo con aplicación de test de Chi-cuadrado para significancia estadística (p<0,05)...

INTRODUCTION: The use of the endoscope and the advance of the technology has facilitated the extension of the traditional limits in the surgery of skull base, nasal cavity and paranasal sinuses. OBJECTIVE: To describe the epidemiologic, clinical and histopathological findings in patients intervened under advanced endoscopic surgery in an Otorhinolaryngology unit in 40 months. MATERIAL AND METHODS: Retrospective study. Surgical protocols and medical records were reviewed. A descriptive univariate analysis was performed with the application of Chi-square test for statistical significance (p <0.05)...

INTRODUÇÃO: O uso do endoscópio junto com o avanço da tecnologia facilitou a extensão dos limites tradicionais na cirurgia da base do crânio, a cavidade nasal e os seios paranasais. OBJETIVO: Descrever os achados epidemiológicos, clínicos e histopatológicos em pacientes submetidos a cirurgia endoscópica avançada em uma unidade de Otorrinolaringologia em 40 meses. MATERIAL E MÉTODOS: Estudo retrospectivo. Os protocolos cirúrgicos e os registros médicos foram revisados. Uma análise descritiva univariada foi realizada com a aplicação do teste Qui-quadrado para significância estatística...

Novel aspects in histopathology of the pituitary gland. / Aspectos novedosos en histopatología de la hipófisis.

The sellar and parasellar region is a complex anatomical area in which several diseases may develop. The pituitary gland may be affected by a wide range of conditions having similar clinical characteristics. Diagnosis of these lesions requires a multidisciplinary approach including, in addition to clinical, laboratory, imaging, and surgical findings, histological diagnosis of pituitary adenomas to guide therapeutic management. As the result of development in recent years of new immunohistochemical techniques, histopathological classification has become more complex and wide, and not only continues to be the gold standard in diagnosis, but also has prognostic implications. The aim of this review is to provide a clear and simple update of the main concepts of histological diagnosis of the most common pituitary conditions, especially for professionals in direct contact with such diseases.

Asociación de craneofaringioma y síndrome de Klinefelter en la transición puberal: un desafío diagnóstico / Craniopharyngioma and Klinefelter syndrome during the pubertal transition: A diagnostic challenge

Los craneofaringiomas son de los tumores hipofisarios más frecuentes en la niñez y, sea por su evolución o por el tratamiento que requieren, pueden comprometer el desarrollo puberal. El síndrome de Klinefelter es la causa más frecuente de hipogonadismo hipergonadotrópico en el varón. La presentación concomitante de ambas entidades es extremadamente baja (1/10(9)) y plantea un interrogante acerca de una probable asociación fisiopatológica. Se presenta el caso de un paciente belga de 18 años, con diagnóstico de craneofaringioma en la niñez y panhipopituitarismo luego del tratamiento quirúrgico y radioterápico. Al llegar a los 14 años, se inició la inducción puberal con gonadotropinas. Ante la falta de respuesta clínica, se completó una evaluación genética, que evidenció, de manera homogénea, una trisomía XXY. La falta de respuesta al tratamiento de inducción con gonadotropina exógena reveló la asociación de hipogonadismo primario y secundario, que demostró la importancia del seguimiento multidisciplinario que estos pacientes requieren.

Craniopharyngioma is the most common pituitary tumor in childhood. It can compromise the pubertal development because of its evolution or treatment. Syndrome of Klinefelter is the most common cause of hipergonadotrophic hypogonadism in males. The concomitant presentation of both entities is extremely low (1/10(9)) and the pathophysiological association is questionned. We present the case of a 18-year-old Belgian patient. He had a diagnosis of craniopharyngioma in childhood and he presented with panhypopituitarism after radiotherapy and surgical treatment. At the age of 14, he started pubertal induction with gonadotropin therapy without clinical response. A genetic evaluation confirmed a homogeneous 47, XXY karyotype. Failure of exogenous gonadotropin therapy revealed the hidden association of primary and secondary hypogonadism, demonstrating the importance of the followup and a multidisciplinary approach in these patients.

Evaluación nutricional de la población con antecedente de craneofaringioma infantil en el Hospital "Prof. Dr. Juan P. Garrahan" / Nutritional assessment of a population with a history of childhood craniopharyngioma seen at Hospital "Prof. Dr. Juan P. Garrahan"

Introducción. los craneofaringiomas son malformaciones histológicamente benignas entre el hipotálamo y la hipófisis que pueden afectar la secreción hormonal clave en la regulación endócrina y modulación de la saciedad. Si bien se trata de una enfermedad relativamente benigna, la combinación de obesidad grave de origen hipotalámico y las comorbilidades asociadas a ella disminuyen la calidad de vida. Objetivo. Evaluar el estado nutricional de pacientes operados de craneofaringioma infantil. Población y métodos. Pacientes menores de 21 años al momento del estudio que requirieron cirugía por craneofaringioma en el Hospital de Pediatría Garrahan y que firmaron el consentimiento informado. Se realizó la evaluación antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta e ingesta energética. Se determinó la resistencia a la insulina y la dislipidemia. Resultados. Se incluyeron 39 pacientes; 41%, normopeso y 59%, obesos. El 68% de los pacientes presentó distribución grasa central; 40%, resistencia a la insulina; y 32%, dislipidemia. No se encontraron diferencias significativas en la presencia de resistencia a la insulina, dislipidemia, gasto energético en reposo ni en la ingesta entre obesos vs. normopeso. El 77% de los pacientes obesos presentó bajo gasto energético independiente del porcentaje de masa magra (62 ± 2,7% vs. 61,2 ± 1,8% de gasto energético en reposo normal vs. bajo; p 0,8). Conclusiones. El 59% de la población estudiada presentó obesidad. No se encontraron diferencias significativas en complicaciones metabólicas entre pacientes obesos y normopeso. Se observó menor gasto energético independiente del porcentaje de masa magra y similar ingesta energética.

Introduction. Craniopharyngiomas are histologically benign malformations located between the pituitary and hypothalamus that may affect key hormone secretion for endocrine regulation and satiety modulation. Although this is a relatively benign disease, the combination of severe hypothalamic obesity and associated comorbidities results in a reduced quality of life. Objective. To assess the nutritional status of patients after craniopharyngioma surgery. population and Methods. Patients younger than 21 years old at the time of the study who required craniopharyngioma surgery at Hospital de Pediatria Garrahan and who signed an informed consent. Anthropometric characteristics, body composition by impedance analysis, energy expenditure by indirect calorimetry and energy intake were assessed. Insulin resistance and dyslipemia were estimated. Results. A total of 39 patients were included; 41% had a normal weight and 59% were obese. Overall, 68% of patients had a central fat distribution; 40% had insulin resistance; and 32%, dyslipemia. No significant differences were observed in terms of insulin resistance, dyslipemia, energy expenditure at rest, or energy intake between normal weight and obese patients. Among obese patients, 77% had a low energy expenditure, regardless of their percentage of lean body mass (62 ± 2.7% versus 61.2 ± 1.8% of normal versus low energy expenditure at rest; p = 0.8). Conclusions. A total of 59% of the studied population was obese. No significant differences were observedinterms ofmetabolic complications between normal weight and obese patients. A lower energy expenditure was observed, regardless of the lean body mass percentage and a similar energy intake.

[Ectopic craniopharyngioma and Gardner's syndrome: Case report and literature review]. / Craneofaringioma ectópico y síndrome de Gardner: a propósito de un caso y revisión de la literatura.

INTRODUCTION: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. CASE REPORT: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset. The examination showed mild right VII and VI cranial nerves paresis. Magnetic resonance imaging of the brain demonstrated a rounded solid and cystic lesion with well-defined contours of about 2cm in diameter filling the fourth ventricle. The patient underwent a posterior fossa craniotomy using a telovelar approach with complete removal of the tumour implanted at roof level of the fourth ventricle. The final histology of the tumour was reported as adamantinomatous craniopharyngioma. CONCLUSION: Craniopharyngioma may appear in another location other than the suprasellar region. Its atypical location may be related to Gardner syndrome by still unknown pathogenic mechanisms.

Gasto energético, composición corporal, trastornos metabólicos e ingesta energética en pacientes con antecedentes de craneofaringioma / Energy expenditure, body composition, metabolic disorders, and energy intake in patients with a history of craniopharyngioma

Introducción: Los craneofaringiomas son malformaciones histológicamente benignas que se sitúan entre el hipotálamo y la hipófisis, zonas con un rol determinante en la modulación de la saciedad. Aun siendo tumores benignos, presentan una considerable morbilidad. La obesidad está presente hasta en un 52% de los pacientes. Objetivo: evaluar factores de riesgo cardiovascular, composición corporal y gasto energético en pacientes con craneofaringioma, y compararlos con un grupo de obesos multifactoriales. Material y métodos: Se incluyeron todos los pacientes con resección quirúrgica de craneofaringioma, menores de 21 años, en seguimiento en nuestro centro entre mayo 2012 hasta abril 2013 que aceptaron participar por medio del consentimiento informado. Se realizó valoración antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta y valoración de ingesta energética y de macronutrientes. Se determinó resistencia a la insulina (HOMA-IR) y dislipemia. Se comparó a los pacientes con craneofaringioma con obesidad, con un grupo de pacientes con obesidad multifactorial. Resultados: se estudiaron 39 pacientes. El 59% era obeso y presentó significativamente menor% de masa magra (62.4 vs 67.5 p=0.01) y mayor% de masa grasa (37.5 vs 32.5 p=0.01) comparados con los obesos multifactoriales. No se encontró diferencias en el compromiso metabólico entre los obesos con y sin antecedente de craneofaringioma. Se dividieron los pacientes en tertilos según% de gasto energético para categorizar en gasto bajo vs normal. Se encontró asociación positiva entre% de gasto energético y% de masa magra en obesos multifactoriales (68±1%; en los gasto normal vs 62.6± 1% en los gasto bajo: p 0,04). Sin diferencias dentro de la población de obesos con antecedente de craneofaringioma (62±2.7 en los gasto normal/alto vs 61.2±1.8% en los gasto bajo: p 0,8). El gasto energético basal (REE) fue menor en los pacientes con antecedente de craneofaringioma vs obesos multifactoriales, independientemente de la masa magra, lo que sustenta que existirían otros factores que actuarían disminuyendo el gasto energético. No hubo diferencia con respecto a la ingesta en ambos grupos estudiados. Conclusiones: los pacientes con antecedente de craneofaringioma presentan menor gasto energético no relacionado a la masa magra y similar ingesta energética comparado con obesos multifactoriales. No hubo diferencias en el compromiso metabólico entre los obesos con y sin antecedentes de craneofaringioma (AU)

Introduction: Craniopharyngiomas are histologically benign malformations located between hypothalamus and the pituitary gland, areas that play an important role in satiety modulation. Although the tumors are benign, they may cause significant morbidity. Obesity is found in up to 52% of patients. Aim: To assess cardiovascular risk factors, body composition, and energy expenditure in patients with craniopharyngioma, and to compare them to results in a group of children with multifactorial obesity. Material and methods: All patients who underwent surgical resection of craniopharyngioma, younger than 21 years of age, who were being followed-up at our center between May 2012 and April 2013 who gave their informed consent to participate were enrolled in the study. Anthropometric measurements, body composition with impedanciometer, energy expenditure with indirect calorimetry, and energy and macronutrient intake were evaluated. Insulin resistance (HOMA-IR) and dyslipidemia were determined. Patients with craniopharyngioma associated with obesity were compared to patients with multifactorial obesity. Results: Of 39 patients studied, 59% were obese and a significantly lower percentage of lean mass (62.4 vs 67.5 p=0.01) and a higher percentage of fat mass (37.5 vs 32.5 p=0.01) compared to multifactorial obese subjects. No differences were found in metabolic involvement between obese subjects with and those without a history of craniopharyngioma. Patients were divided into tertiles according to percentage of energy expenditure to categorize low versus normal expenditure. A positive correlation was found between percentage of energy expenditure and lean mass percentage in subjects with multifactorial obesity (68±1%; in those with normal energy expenditure versus 62.6±1% in those with low energy expenditure: p 0.04). No difference was found within the group of obese patients with a history of craniopharyngioma (62±2.7 in those with normal/high expenditure versus 61.2±1.8% in those with low expenditure: p 0.8). Baseline energy expenditure (BEE) was lower in craniopharyngioma patients than in those with multifactorial obesity, regardless of lean mass percentage, supporting the hypothesis that other factors may be involved in the decrease of energy expenditure. There was no difference in the food intake between both groups. Conclusions: Patients with a history of craniopharyngioma had a lower energy expenditure unrelated to lean mass and a similar energy intake compared to subjects with multifactorial obesity. No differences were found in metabolic involvement between obese subject with and those without a history of craniopharyngioma (AU)

[Practice guideline for diagnosis and treatment of craniopharyngioma and parasellar tumors of the pituitary gland]. / Guía práctica del manejo y tratamiento de los craneofaringiomas y otras lesiones paraselares.

Craniopharyngiomas are rare, locally aggressive epithelial tumors usually located in the sellar and suprasellar region. Diagnosis of craniopharyngioma is usually suggested by clinical and radiological findings that should be confirmed histologically. Surgery is the treatment of choice for most patients. The goal of surgery is to relieve compressive symptoms and to remove as much tumor as safely possible. Radiation therapy is the usual treatment to control postoperative tumor remnants and local recurrences. Parasellar lesions are low prevalent lesions and include neoplastic, inflammatory, infectious, developmental, and vascular diseases. Both their diagnosis and treatment depend on the type of lesion.

Craneofaringioma en la tercera edad / Craniopharyngioma in the elderly

Los craneofaringiomas representan del 6 al 10 por ciento de las neoplasias intracraneales en la infancia y el 30 por ciento en adultos; infrecuentes en la tercera edad. Las manifestaciones clínicas diversas (síntomas visuales, endocrinos y neurológicos de la esfera psíquica superior) dificultan su diagnóstico en la tercera edad, de ahí la importancia de la presentación de este caso. Paciente masculino de la séptima década de la vida, con cefalea y déficit visual progresivos. Los estudios neuroimageológicos avalaron la cirugía. La evolución fue satisfactoria. El examen histopatológico confirmó el diagnóstico de craneofaringioma. Se trata de un tumor benigno intracraneal infrecuente en la tercera edad, que debe tenerse en cuenta en pacientes con estos síntomas (AU)

Craniopharyngiomas represent 6 to 10 percent of the intracranial tumors in childhood and 30 percent in adults; they are rare in the elderly. Their diverse clinical manifestations, such as visual, endocrine and superior psychic sphere symptoms make it difficult to be diagnosed in the elderly, hence the importance of the presentation of this case, which is about a male patient in his seventies, suffering from headache and progressive visual deficit. The neuro-imaginal studies supported his surgery. His evolution was satisfactory. The histopathologic examination confirmed the diagnosis of craniopharyngioma, an infrequent intracranial benign tumor in the elderly, which has to be taken into consideration in patients showing those symptoms (AU)

[The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part II: Anatomical and neuroradiological evidence to define triple-axis topography and its usefulness in predicting individual surgical risk]. / Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual.

INTRODUCTION AND OBJECTIVES: This study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal. MATERIAL AND METHODS: An extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n=145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n=224 cases) and of non-operated cases reported in autopsies (n=201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n=130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury. RESULTS: The 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables -patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle- makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). CONCLUSIONS: A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.