Orbitotomía supero medial: nuevos límites en el abordaje endonasal expandido transetmoidal

Introducción: Los límites de los abordajes endonasales endoscópicos expandidos (AEEE) no están delimitados y continúan en constante evolución. El límite de la extensión lateral en el plano coronal del AEEE transetmoidal descrito hasta el momento viene determinado por la unión del techo de las fóveas etmoidales con la lámina papirácea. Objetivos: Este trabajo se basa en la hipótesis fundamental de que la resección de lesiones situadas en la base craneal anterior, concretamente en la porción formada por el hueso frontal y asentadas más allá de las láminas papiráceas, pueden ser abordadas de forma completamente endonasal endoscópica. La hipótesis operativa considera que es posible mediante la realización de una etmoidectomía más una orbitotomía superomedial el abordaje a estas lesiones, así como la obtención de medidas en el plano coronal de cuan lateral se puede extender la resección de forma totalmente endoscópica en las diferentes porciones de la base craneal anterior. Material y métodos: Estudio prospectivo realizado sobre 15 especímenes cadavéricos inyectados con silicona coloreada. Se realizaron 30 orbitotomías supero mediales tras previa etmoidectomía total bilateral de forma totalmente endoscópica. Posteriormente se practicó sobre los mismos especímenes cadavéricos una craniotomía bicoronal para resecar ambos lóbulos frontales y poder realizar mediciones precisas del abordaje endonasal realizados endoscópicamente sobre la fosa craneal anterior. Para poder lidiar con el diferente tamaño de los especímenes se tomaron fotografías digitales de cada espécimen y mediante análisis informático se dividió la fosa craneal anterior de cada cabeza en 5 zonas de igual tamaño entre sí (Zona 0 o Zona sinusal, Zona 1 o Zona presinusal, Zona 2 o Zona etmoidal anterior, Zona 3 o Zona interetmoidal y Zona 4 o Zona etmoidal posterior). Cada una de las zonas representaba de esta manera el 20% del total de la superficie basicraneal anterior. Las áreas fueron siempre proporcionales a la longitud de cada fosa craneal. Resultados: La realización de la orbitotomía supero medial endoscópica (resección de la porción medial y superior de la órbita mediante cirugía totalmente endoscópica) resultó viable y reproductible. En 60% de las ocasiones el desgarro de la duramadre al trabajar en ángulos de trabajo forzados fue la causa de la detención de la técnica. La zona donde mayor extensión lateral se pudo alcanzar corresponde a la situada entre ambas arterias etmoidales con una media de 8 mm y un diámetro medio de 45,40±7 mm. La extensión lateral desde la línea media en la zona más próxima al nervio óptico izquierdo fue significativamente mayor (p=0,014) que en el lado derecho (21±4,1 mm vs 19,2±3 mm). La extensión lateral en el lado derecho desde la lámina papirácea en la porción más próxima al seno frontal fue significativa mayor (p=0,005) que en el lado izquierdo (2,93±2 mm vs 5,5±2 mm). Conclusiones: Muchas de las lesiones situadas en la base de craneal anterior con amplia base de implantación que no se limitan a la línea media y se extienden lateralmente más allá de las láminas papiráceas pueden ser abordadas de forma completamente endonasal endoscópica.The limits of the endonasal endoscopic expanded approaches are not yet defined. The lateral extension in the coronal plane of the transethmoidal approach described so far is determined by the papyracea lamina. Objectives: This work is based on the main hypothesis that the resection of lesions located in the anterior cranial base, beyond the papyracea lamina, can be approach fully endonasal endoscopic way. The working hypothesis considers it possible performing an ethmoidectomy plus a superomedial orbitotomy approach these lesions, as well as obtaining measures in the coronal plane of the laterality you can get via totally endoscopic in the different portions of the anterior cranial base. The secondary hypothesis contemplates that knowledge of the limits of this approach can help in the future to the skull base surgeons to opt for endoscopic endonasal surgery or transcranial open approach to operate a lesion located in the anterior cranial base. Methods: Prospective study of 15 cadaveric specimens injected with colored silicone. 30 superomedial orbitotomies were performed endoscopicaly after previous bilateral ethmoidectomy. Later, on the same cadaveric specimens, a bicoronal craniotomy was done and both frontal lobes were resected. Precise measurements of the endoscopic endonasal approach previously performed on the anterior cranial fossa were taken. To deal with the different size of the specimens digital photographs of each specimen were taken. By computer analysis of the anterior cranial fossa each head was divided into 5 zones of equal size each other (Zone 0 or Sinusal zone, Zone 1 or Presinusal zone, Zone 2 or Anterior Ethmoidal zone, Zone 3 or Interethmoidal zone and Zone 4 or Posterior Ethmoidal zone). Each of the areas represented 20% of the basicranial surface. Results: Perform a superomedial orbitotomy (endoscopic resection of the medial and upper portion of the orbit by endoscopic endonasal surgery) was feasible and reproducible. In 60% of cases tearing of the duramater was the cause of the arrest of the technique. The area where greater lateral extension could be achieved corresponds to the Zone 3 or Interethmoidal zone, between both ethmoidal arteries, with an average of 8 mm and an average diameter of 45.40 ± 7 mm. The lateral extension from the midline, in the area closest to the left optic nerve, was significantly higher (p = 0.014) than the right side (21 ± 4.1 mm vs 19.2 ± 3 mm). The lateral extension on the right side from the lamina papyracea, in the nearest portion to the frontal sinus, was significantly higher (p = 0.005) than in the left (2.93 vs 5.5 ± 2 mm ± 2 mm). Conclusions: Many mass lesions located in the anterior cranial not limited to the midline and extending laterally beyond the papyracea laminas can be appoached completely via endoscopic endonasal transethmoidal adding a superomedial orbitotomy

A Silent Corticotroph Pituitary Carcinoma: Lessons From an Exceptional Case Report

Nowadays, neither imaging nor pathology evaluation can accurately predict the aggressiveness or treatment resistance of pituitary tumors at diagnosis. However, histological examination can provide useful information that might alert clinicians about the nature of pituitary tumors. Here, we describe our experience with a silent corticothoph tumor with unusual pathology, aggressive local invasion and metastatic dissemination during follow-up. We present a 61-year-old man with third cranial nerve palsy at presentation due to invasive pituitary tumor. Subtotal surgical approach was performed with a diagnosis of silent corticotroph tumor but with unusual histological features (nuclear atypia, frequent multinucleation and mitotic figures, and Ki-67 labeling index up to 70%). After a rapid regrowth, a second surgical intervention achieved successful debulking. Temozolomide treatment followed by stereotactic fractionated radiotherapy associated with temozolomide successfully managed the primary tumor. However, sacral metastasis showed up 6 months after radiotherapy treatment. Due to aggressive distant behavior, a carboplatine-etoposide scheme was decided but the patient died of urinary sepsis 31 months after the first symptoms. Our case report shows how the presentation of a pituitary tumor with aggressive features should raise a suspicion of malignancy and the need of follow up by multidisciplinary team with experience in its management. Metastases may occur even if the primary tumor is well controlled.This work was supported by grants from the ISCIII-Subdirección General de Evaluación y Fomento de la Investigación co-funded with Fondos FEDER (PI16/00175 to AS-M and DC) and the Sistema Andaluz de Salud (A-0003-2016 and A-0006-2017 to AS-M, C-0015-2014 and RC-0006-2018 to DC)

Sex Hormone Receptor Expression in Craniopharyngiomas and Association with Tumor Aggressiveness Characteristics

Craniopharyngiomas (CPs) are rare tumors of the sellar and suprasellar regions of embryonic origin. The primary treatment for CPs is surgery but it is often unsuccessful. Although CPs are considered benign tumors, they display a relatively high recurrence rate that might compromise quality of life. Previous studies have reported that CPs express sex hormone receptors, including estrogen and progesterone receptors. Here, we systematically analyzed estrogen receptor α (ERα) and progesterone receptor (PR) expression by immunohistochemistry in a well-characterized series of patients with CP (n = 41) and analyzed their potential association with tumor aggressiveness features. A substantial proportion of CPs displayed a marked expression of PR. However, most CPs expressed low levels of ERα. No major association between PR and ERα expression and clinical aggressiveness features was observed in CPs. Additionally, in our series, β-catenin accumulation was not related to tumor recurrence. View Full-TextThis work was supported by grants from the ISCIII-Subdirección General de Evaluación y Fomento de la Investigación co-funded with Fondos FEDER (PI16/00175 to A.S-M. and D.A.C.) and the Sistema Andaluz de Salud (A-0006-2017 and A-0055-2018 to A.S-M, RC-0006-2018 to D.A.C.)

Refining the anatomic boundaries of the endoscopic endonasal transpterygoid approach: the "VELPPHA area" concept.

The endoscopic endonasal transpterygoid route has been widely evaluated in cadavers, and it is currently used during surgery for specific diseases involving the lateral skull base. Identification of the petrous segment of the internal carotid artery (ICA) is a key step during this approach, and the vidian nerve (VN) has been described as a principal landmark for safe endonasal localization of the petrous ICA at the level of the foramen lacerum. However, the relationship of the VN to the ICA at this level is complex as well as variable and has not been described in the pertinent literature. Accordingly, the authors undertook this purely anatomical study to detail and quantify the peri-lacerum anatomy as seen via an endoscopic endonasal transpterygoid pathway. Eight human anatomical specimens (16 sides) were dissected endonasally under direct endoscopic visualization. Anatomical landmarks of the VN and the posterior end of the vidian canal (VC) during the endoscopic endonasal transpterygoid approach were described, quantitative anatomical data were compiled, and a schematic classification of the most relevant structures encountered was proposed. The endoscopic endonasal transpterygoid approach was used to describe the different anatomical structures surrounding the anterior genu of the petrous ICA. Five key anatomical structures were identified and described: the VN, the eustachian tube, the foramen lacerum, the petroclival fissure, and the pharyngobasilar fascia. These structures were specifically quantified and summarized in a schematic acronym-VELPPHA-to describe the area. The VELPPHA area is a dense fibrocartilaginous space around the inferior compartment of the foramen lacerum that can be reached by following the VC posteriorly; this area represents the posterior limits of the transpterygoid approach and, of utmost importance, no neurovascular structures were observed through the VELPPHA area in this study, indicating that it should be a safe zone for surgery in the posterior end of the endoscopic endonasal transpterygoid approach. The VELPPHA area represents the posterior limits of the endoscopic endonasal transpterygoid approach. Early identification of this area can enhance the safety of the endoscopic endonasal transpterygoid approach expanded to the lateral aspect of the skull base, especially when treating patients with poorly pneumatized sphenoid sinuses

Nuestra experiencia en prolactinomas mayores de 60 mm

[EN] Introduction: Giant prolactinomas (tumor size larger than 40 mm) are a rare entity of benign nature. Prolactinomas larger than 60 mm are usually underrepresented in published studies and their clinical presentation, outcomes and management might be different from smaller giant prolactinomas. Patients and methods: We retrospective collected data from patients with prolactinomas larger than 60 mm in maximum diameter and prolactin (PRL) serum levels higher than 21,200 μIU/mL in our series of prolactinomas (283). Data were collected from January 2012 to December 2017. We included three patients with prolactinomas larger than 60 mm. Results: At diagnosis, two patients presented neurological symptoms and one nasal protrusion. All patients received medical treatment with dopamine agonists. No surgical procedure was performed. Median prolactin levels at diagnosis was 108,180 [52,594–514,984] μIU/mL. Medical treatment achieved a marked reduction (>99%) in prolactin levels in all cases. Tumor size reduction (higher than 33%) was observed in all cases. In one patient cerebrospinal fluid (CSF) leak was observed after tumor shrinkage. Conclusions: Dopamine agonists appear to be an effective and safe first-line treatment in prolactinomas larger than 60 mm even in life-threatening situations. More studies with a higher number of patients are necessary to obtain enough data to make major recommendations.[ES] Introducción: Los prolactinomas gigantes (de tamaño superior a 40 mm) son una entidad rara de naturaleza benigna. Los prolactinomas mayores de 60 mm suelen estar infrarrepresentados en los estudios publicados, y su presentación clínica, resultados y tratamiento podrían ser diferentes de los de prolactinomas gigantes más pequeños. Pacientes y métodos: Recogimos retrospectivamente datos de pacientes con prolactinomas de más de 60 mm de diámetro máximo y con concentraciones séricas de prolactina (PRL) superiores a 21.200 μIU/ml de nuestra serie de prolactinomas (283). Los datos se recogieron entre enero de 2012 y diciembre de 2017. Se incluyeron 3 pacientes con prolactinomas mayores de 60 mm. Resultados: En el momento del diagnóstico, 2 pacientes presentaban síntomas neurológicos, y uno protrusión nasal. Todos los pacientes recibieron tratamiento médico con agonistas dopaminérgicos. No se realizó ninguna intervención quirúrgica. La mediana de las concentraciones de PRL al diagnóstico fue de 108.180 (52.594-514.984) μIU/ml. El tratamiento médico logró una reducción notable (> 99%) de los valores de prolactina en todos los casos. En todos los casos se observó una reducción del tamaño del tumor (superior al 33%). En un paciente se observó una fuga de líquido cefalorraquídeo (LCR) tras la reducción del tumor. Conclusión: Los agonistas dopaminérgicos parecen ser un tratamiento de primera línea eficaz y seguro en los prolactinomas mayores de 60 mm incluso en situaciones peligrosas para la vida. Se necesitan más estudios con un mayor número de pacientes para obtener datos suficientes para hacer recomendaciones importantes

Clinical Applicability of the Sellar Barrier Concept in Patients with Pituitary Apoplexy: Is It Possible?

There is evidence of association between sellar barrier thickness and intraoperative cerebrospinal fluid (CSF) leakage, impacting the postoperative prognosis of the patients. The aim of this study is to analyze the clinical applicability of the sellar barrier concept in a series of operated patients with pituitary apoplexy (PA). A retrospective study was conducted including 47 patients diagnosed with PA who underwent surgical treatment through a transsphenoidal approach. Brain magnetic resonance imaging (MRI) of the patients were evaluated and classified utilizing the following criteria: strong barrier (greater than 1 mm), weak barrier (less than 1 mm), and mixed barrier (less than 1 mm in one area and greater than 1 mm in another). The association between sellar barrier types and CSF leakage was analyzed, both pre- and intraoperatively. The preoperative MRI classification identified 10 (21.28%) patients presenting a weak sellar barrier, 20 patients (42.55%) with a mixed sellar barrier, and 17 patients (36.17%) exhibiting a strong sellar barrier. Preoperative weak and strong sellar barrier subtypes were associated with weak (p ≤ 0.001) and strong (p = 0.009) intraoperative sellar barriers, respectively. Strong intraoperative sellar barrier subtypes reduced the odds of CSF leakage by 86% (p = 0.01). A correlation between preoperative imaging and intraoperative findings in the setting of pituitary apoplexy has been observed

Quantitative Analysis of Somatostatin and Dopamine Receptors Gene Expression Levels in Non-functioning Pituitary Tumors and Association with Clinical and Molecular Aggressiveness Features

The primary treatment for non-functioning pituitary tumors (NFPTs) is surgery, but it is often unsuccessful. Previous studies have reported that NFPTs express receptors for somatostatin (SST1-5) and dopamine (DRDs) providing a rationale for the use of dopamine agonists and somatostatin analogues. Here, we systematically assessed SST1-5 and DRDs expression by real-time quantitative PCR (RT-qPCR) in a large group of patients with NFPTs (n = 113) and analyzed their potential association with clinical and molecular aggressiveness features. SST1-5 expression was also evaluated by immunohistochemistry. SST3 was the predominant SST subtype detected, followed by SST2, SST5, and SST1. DRD2 was the dominant DRD subtype, followed by DRD4, DRD5, and DRD1. A substantial proportion of NFPTs displayed marked expression of SST2 and SST5. No major association between SSTs and DRDs expression and clinical and molecular aggressiveness features was observed in NFPTs