A pure extradural hemangioblastoma mimicking a dumbbell nerve sheath tumor in cervical spine: illustrative case.

BACKGROUND: Spinal extradural hemangioblastomas (HBs) are quite uncommon, with most reported cases involving the thoracic and lumbar areas. Therefore, the presence of a dumbbell-shaped pure extradural cervical HB is exceptional, making preoperative diagnosis particularly challenging. OBSERVATIONS: The authors report a case of a 27-year-old woman who presented to their outpatient clinic with progressive cervicobrachialgia and numbness in the left arm. Magnetic resonance imaging showed a C5-6 intradural extramedullary lesion, and, despite some atypical features, the diagnosis of a possible neurogenic tumor was made. A multidetector computed tomography scan and angiography confirmed the expansion and remodeling of the left neural foramen as well as the highly vascularized nature of the mass. Preoperative embolization of the lesion was performed. Complete tumor resection was accomplished, followed by a C5-6 posterior fusion. Histopathology and immunohistochemistry revealed an HB. LESSONS: HBs should be considered among the differential diagnosis of cervical extradural tumors. Exhaustive preoperative workup and surgical planning are decisive in order to attain gross-total resection with favorable outcomes.

Cervical intramedullary solitary fibrous tumor: Case report and review of the literature.

BACKGROUND: Solitary fibrous tumors (SFTs) are benign tumors derived from mesenchymal tissues that predominantly occur in the pleura. Establishing the diagnosis of these very rare intramedullary spinal lesions, with no clear-cut pathognomonic radiographic characteristics, is particularly challenging. CASE DESCRIPTION: Two males, 30 and 41 years of age, presented with progressive cervical myelopathies attributed to a cervical intramedullary exophytic tumor with associated spinal cord edema. One patient showed that the lesion was highly vascularized. Both patients underwent surgical excision of firm, solid, focal, and, particularly in one of them, very vascular/hemorrhagic tumors; at surgery, there was some adherence between the tumors and the cord tissue, but gross-total resections were achieved in both cases, demonstrated on postoperative MR scans. Histological and immunohistochemical findings confirmed the diagnosis of SFT (WHO Grade I). After a 6-month postoperative period, both patients neurologically improved and had no MR evidence of tumor recurrence. CONCLUSION: Intramedullary cervical exophytic SFTs are extremely rare. Although these solid tumors may present with hemorrhagic features and at surgery demonstrate significant adherence to the pial/cord surface, complete surgical resections are feasible resulting in good outcomes.

Prognostic usefulness of ACTH in the postoperative period of Cushing's disease.

OBJECTIVES: To analyze the usefulness of plasma ACTH in predicting CD remission after surgery and to evaluate the prognostic usefulness of ACTH measurement after the cortisol and ACTH nadir (48 h prior to discharge). DESIGN: A prospective study was made of 65 patients with CD operated upon between 2005 and 2016. METHODS: Postsurgery plasma ACTH and cortisol were measured every 6 h, in the absence of corticosteroid coverage. Hydrocortisone was started in the presence of adrenal insufficiency or cortisol <55.2 nmol/L. Plasma ACTH was again determined before discharge. MAIN OUTCOME MEASURE: Usefulness of plasma ACTH in predicting CD remission. RESULTS: Remission at 3 months of CD was achieved in 56 of 65 cases, with late recurrence in 18 of 58 cases. Following resection, the ACTH nadir was significantly lower referred to late remission (2.8 vs 6.5 pmol/L; P = 0.031) and higher for recurrence (2.1 vs 4.8 pmol/L; P < 0.001), and identical results were obtained for the ACTH values before discharge. In the analysis of the ROC curves, nadir and before discharge ACTH values <1.9 pmol/L and <2.6 pmol/L were respectively indicative of early remission (AUC 0.827; P < 0.001); <6.2 pmol/L of remission at 3 months (AUC 0.847; P = 0.001) and >3.2 pmol/L of recurrence (AUC 0.810; P < 0.001) in both ACTH values. A time to ACTH nadir <46 h was indicative of early remission (AUC 0.751; P = 0.001), while a time >39 h was indicative of recurrence (AUC 0.773; P = 0.001). CONCLUSIONS: We propose an ACTH value <3.3 pmol/L as a good long-term prognostic marker in the postoperative period of CD. Reaching the ACTH nadir in less time is associated to a lesser recurrence rate.

Anatomía microquirúrgica en 3D del tracto corticoespinal y de la vía del lemnisco basada en microdisección de fibras y demostración a través de tractografía / 3D microsurgical anatomy of the cortico-spinal tract and lemniscal pathway based on fiber microdissection and demonstration with tractography

Objetivo: Realizar un estudio anatómico de microdisección de fibras y radiológico mediante tractografía basada en tensor de difusión (DTT) para demostrar tridimensionalmente el tracto corticoespinal y el lemnisco medial. Material y métodos: Bajo visión microscópica y con el uso de instrumental microquirúrgico se disecaron y estudiaron 10 hemisferios cerebrales y 15 troncoencéfalos humanos a través de la técnica de microdisección de fibras. Se obtuvieron imágenes de resonancia magnética cerebrales de 15 sujetos sanos, empleando secuencias potenciadas en difusión para el trazado y reproducción mediante DTT del tracto corticoespinal y la vía del lemnisco. Resultados: Se demostraron y describieron anatómicamente el tracto corticoespinal y lemnisco medial en gran parte de sus trayectorias, identificando las relaciones entre sí y con otros haces de sustancia blanca y núcleos de sustancia gris cercanos, especialmente en el troncoencéfalo, con la correspondiente representación mediante DTT. Conclusiones: Mediante la técnica de microdisección se apreció la disposición, arquitectura y organización topográfica general del tracto corticoespinal y lemnisco medial. Este conocimiento ha aportado una perspectiva anatómica única y profunda que ha favorecido la representación y la correcta interpretación de las imágenes de DTT. Esta información debe ser trasladada a la práctica clínica para favorecer el análisis crítico y exhaustivo por parte del cirujano ante posibles lesiones localizadas en el interior del troncoencéfalo y, en consecuencia, mejorar la indicación y planificación quirúrgica, incluyendo la selección preoperatoria de estrategias óptimas y posibles zonas de abordajes a su interior, alcanzando una técnica microquirúrgica más segura y precisa

Objective: To demonstrate tridimensionally the anatomy of the cortico-spinal tract and the medial lemniscus, based on fiber microdissection and diffusion tensor tractography (DTT). Material and methods: Ten brain hemispheres and brain-stem human specimens were dissected and studied under the operating microscope with microsurgical instruments by applying the fiber microdissection technique. Brain magnetic resonance imaging was obtained from 15 healthy subjects using diffusion-weighted images, in order to reproduce the cortico-spinal tract and the lemniscal pathway on DTT images. Results: The main bundles of the cortico-spinal tract and medial lemniscus were demonstrated and delineated throughout most of their trajectories, noticing their gross anatomical relation to one another and with other white matter tracts and gray matter nuclei the surround them, specially in the brain-stem; together with their corresponding representation on DTT images. Conclusions: Using the fiber microdissection technique we were able to distinguish the disposition, architecture and general topography of the cortico-spinal tract and medial lemniscus. This knowledge has provided a unique and profound anatomical perspective, supporting the correct representation and interpretation of DTT images. This information should be incorporated in the clinical scenario in order to assist surgeons in the detailed and critic analysis of lesions located inside the brain-stem, and therefore, improve the surgical indications and planning, including the preoperative selection of optimal surgical strategies and possible corridors to enter the brainstem, to achieve safer and more precise microsurgical technique

3D microsurgical anatomy of the cortico-spinal tract and lemniscal pathway based on fiber microdissection and demonstration with tractography. / Anatomía microquirúrgica en 3D del tracto corticoespinal y de la vía del lemnisco basada en microdisección de fibras y demostración a través de tractografía.

OBJECTIVE: To demonstrate tridimensionally the anatomy of the cortico-spinal tract and the medial lemniscus, based on fiber microdissection and diffusion tensor tractography (DTT). MATERIAL AND METHODS: Ten brain hemispheres and brain-stem human specimens were dissected and studied under the operating microscope with microsurgical instruments by applying the fiber microdissection technique. Brain magnetic resonance imaging was obtained from 15 healthy subjects using diffusion-weighted images, in order to reproduce the cortico-spinal tract and the lemniscal pathway on DTT images. RESULTS: The main bundles of the cortico-spinal tract and medial lemniscus were demonstrated and delineated throughout most of their trajectories, noticing their gross anatomical relation to one another and with other white matter tracts and gray matter nuclei the surround them, specially in the brain-stem; together with their corresponding representation on DTT images. CONCLUSIONS: Using the fiber microdissection technique we were able to distinguish the disposition, architecture and general topography of the cortico-spinal tract and medial lemniscus. This knowledge has provided a unique and profound anatomical perspective, supporting the correct representation and interpretation of DTT images. This information should be incorporated in the clinical scenario in order to assist surgeons in the detailed and critic analysis of lesions located inside the brain-stem, and therefore, improve the surgical indications and planning, including the preoperative selection of optimal surgical strategies and possible corridors to enter the brainstem, to achieve safer and more precise microsurgical technique.

Anatomía de los pedúnculos cerebelosos en 3D basada en microdisección de fibras y demostración a través de tractografía / 3D anatomy of cerebellar peduncles based on fibre microdissection and a demonstration with tractography

Objetivo: Realizar un estudio anatómico de microdisección de fibras y radiológico mediante tractografía basada en tensor de difusión (DTT) para demostrar tridimensionalmente los pedúnculos cerebelosos superiores, medios e inferiores. Material y métodos: Bajo visión microscópica y con el uso de instrumental microquirúrgico en el laboratorio, se disecaron 15 troncoencéfalos, 15 hemisferios cerebelosos y 5 hemisferios cerebrales humanos, entre julio de 2014 y julio de 2015. Se obtuvieron imágenes de resonancia magnética cerebrales realizas a 15 sujetos sanos entre julio y diciembre de 2015, empleando secuencias potenciadas en difusión para el trazado de los pedúnculos cerebelosos y su reproducción mediante DTT. Resultados: Se demostraron y describieron anatómicamente las principales fibras de los pedúnculos cerebelosos a lo largo de gran parte de su trayectoria en el cerebelo y troncoencéfalo, identificando las relaciones entre sí y con otros haces de sustancia blanca y núcleos de sustancia gris que los rodean, con la correspondiente representación mediante DTT. Conclusiones: Mediante la técnica de microdisección se apreció la disposición, arquitectura y organización topográfica general de los pedúnculos cerebelosos. Este conocimiento ha aportado una perspectiva anatómica única y profunda que ha favorecido la representación y correcta interpretación de las imágenes de DTT. Esta información debe ser trasladada a la práctica clínica para favorecer el análisis crítico y exhaustivo por parte del cirujano ante la presencia de lesiones que puedan localizarse cercanas a este grupo de haces en el cerebelo y/o troncoencéfalo, y, en consecuencia, mejorar la planificación quirúrgica y alcanzar una técnica microquirúrgica más segura y precisa

Objective: To perform an anatomical and radiological study, using fibre microdissection and diffusion tensor tractography (DTT), to demonstrate the three-dimensionality of the superior, middle and inferior cerebellar peduncles. Material and methods: A total of 15 brain-stem, 15 cerebellar hemispheres, and 5 brain hemispheres were dissected in the laboratory under the operating microscope with microsurgical instruments between July 2014 and July 2015. Brain magnetic resonance imaging was obtained from 15 healthy subjects between July and December of 2015, using diffusion-weighted images, in order to reproduce the cerebellar peduncles on DTT. Results: The main bundles of the cerebellar peduncles were demonstrated and delineated along most of their trajectory in the cerebellum and brain-stem, noticing their overall anatomical relationship to one another and with other white matter tracts and the grey matter nuclei the surround them, with their corresponding representations on DTT. Conclusions: The arrangement, architecture, and general topography of the cerebellar peduncles were able to be distinguished using the fibre microdissection technique. This knowledge has given a unique and profound anatomical perspective, supporting the correct representation and interpretation of DTT images. This information should be incorporated in the clinical scenario in order to assist surgeons in the detailed and critical analysis of lesions that may be located near these main bundles in the cerebellum and/or brain-stem, and therefore, improve the surgical planning and achieve a safer and more precise microsurgical technique

[3D anatomy of cerebellar peduncles based on fibre microdissection and a demonstration with tractography]. / Anatomía de los pedúnculos cerebelosos en 3D basada en microdisección de fibras y demostración a través de tractografía.

OBJECTIVE: To perform an anatomical and radiological study, using fibre microdissection and diffusion tensor tractography (DTT), to demonstrate the three-dimensionality of the superior, middle and inferior cerebellar peduncles. MATERIAL AND METHODS: A total of 15 brain-stem, 15 cerebellar hemispheres, and 5 brain hemispheres were dissected in the laboratory under the operating microscope with microsurgical instruments between July 2014 and July 2015. Brain magnetic resonance imaging was obtained from 15 healthy subjects between July and December of 2015, using diffusion-weighted images, in order to reproduce the cerebellar peduncles on DTT. RESULTS: The main bundles of the cerebellar peduncles were demonstrated and delineated along most of their trajectory in the cerebellum and brain-stem, noticing their overall anatomical relationship to one another and with other white matter tracts and the grey matter nuclei the surround them, with their corresponding representations on DTT. CONCLUSIONS: The arrangement, architecture, and general topography of the cerebellar peduncles were able to be distinguished using the fibre microdissection technique. This knowledge has given a unique and profound anatomical perspective, supporting the correct representation and interpretation of DTT images. This information should be incorporated in the clinical scenario in order to assist surgeons in the detailed and critical analysis of lesions that may be located near these main bundles in the cerebellum and/or brain-stem, and therefore, improve the surgical planning and achieve a safer and more precise microsurgical technique.

Abceso hipofisario: presentación de 2 casos y revisión de la literatura / Pituitary abscess: Report of two cases and literature review

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Factores pronósticos de remisión a largo plazo tras cirugía transesfenoidal en la enfermedad de Cushing / Predictors of long-term remission after transsphenoidal surgery in Cushing's disease

En el manejo de la enfermedad de Cushing (EC) no existe un consenso sobre los criterios de remisión ni sobre la definición de recurrencia en la literatura, por lo que las seriesno son comparables. Se ha descrito que la tasa de recurrencia en la EC oscila entre 5-25% en el seguimiento a largo plazo. Dentro de los factores pronósticos de remisión a largo plazo podemos diferenciar: 1) factores dependientes del adenoma (agresividad, tamaño, identificación preoperatoria por técnicas de imagen); 2) factores dependientes de la cirugía, donde destaca por su importancia la experiencia del neurocirujano; 3) factores clínicos, siendo la dependencia del tratamiento glucocorticoideo y su duración los más demostrados; y 4) factores bioquímicos. Dentro de estos últimos queda bien documentado en la literatura que un nadir indetectable de cortisol, al menos inferior a 2 mcg/dL, en el postoperatorio predice la remisión de la enfermedad pero, incluso en estos casos, no puede excluirse la recidiva, lo que obliga al seguimiento de por vida en estos pacientes. Presentamos los resultados preliminares de los primeros 20 pacientes intervenidos en el Hospital Universitario de la Ribera utilizando el nadir de cortisol inferior a 2 mcg/dL

There is no consensus on the remission criteria for Cushing's disease or on the definition of disease recurrence after transsphenoidal surgery, and comparison of the different published series is therefore difficult. A long-term recurrence rate of Cushing's disease ranging from 2%-25% has been reported. Predictors of long-term remission reported include: 1) adenoma-related factors (aggressiveness, size, preoperative identification in MRI), 2) surgery-related factors, mainly neurosurgeon experience, 3) clinical factors, of which dependence on and duration of glucocorticoid treatment are most important, and 4) biochemical factors. Among the latter, low postoperative cortisol levels, less than 2 mcg/dL predict for disease remission. However, even when undetectable plasma cortisol levels are present, long-term recurrence may still occur and lifetime follow-up is required. We report the preliminary results of the first 20 patients with Cushing's disease operated on at our hospital using nadir cortisol levels less than 2 mcg/dl as remission criterion

[Predictors of long-term remission after transsphenoidal surgery in Cushing's disease]. / Factores pronósticos de remisión a largo plazo tras cirugía transesfenoidal en la enfermedad de Cushing.

There is no consensus on the remission criteria for Cushing's disease or on the definition of disease recurrence after transsphenoidal surgery, and comparison of the different published series is therefore difficult. A long-term recurrence rate of Cushing's disease ranging from 2%-25% has been reported. Predictors of long-term remission reported include: 1) adenoma-related factors (aggressiveness, size, preoperative identification in MRI), 2) surgery-related factors, mainly neurosurgeon experience, 3) clinical factors, of which dependence on and duration of glucocorticoid treatment are most important, and 4) biochemical factors. Among the latter, low postoperative cortisol levels, less than 2 mcg/dL predict for disease remission. However, even when undetectable plasma cortisol levels are present, long-term recurrence may still occur and lifetime follow-up is required. We report the preliminary results of the first 20 patients with Cushing's disease operated on at our hospital using nadir cortisol levels less than 2 mcg/dl as remission criterion.

Surgical approach in treatment of translation/rotation injuries of the lower cervical spine in 21 patients.

BACKGROUND: The management of lower cervical spine injuries with a dislocation of one or both facet joints and a displacement of a vertebra over the adjacent stills generates considerable controversy. We describe our experience in surgical approach of these injuries. METHODS: We present 21 cases treated between 2003-2010. Neurological status was evaluated with Frankel scale. Diagnosis was done by radiograph (XR), computed tomography (CT) and/or magnetic resonance image (MRI). Cervical traction was placed in 10 cases before surgery. Posterior and/or anterior approach was used for reduction and stabilization. RESULTS: The 21 cases presented were treated by surgery. Posterior approach was initially used in 17 cases and complete reduction was achieved in 13 of them. The 4 cases where we only got a partial reduction, surgery had to be delayed for different reasons. Anterior approach was initially used in 4 of the 21 cases. In 3 of them, reduction was previously obtained by traction and the fourth case anterior approach was used initially due to an important spinal cord compression. Permanent stabilization was achieved in 19 of the 21 cases. In 1 of the other 2 cases an important deformity was detected after the anterior approach. The other case had a minimal progression after a posterior approach with no increase in successive check-ups. In the first 10 cases, we used traction before surgery but reduction was achieved only in 3 of them. As the number of cases increased we rather used posterior approach in the first place, without even trying a preoperative traction. There was no case of neurological deterioration after surgery. CONCLUSION: Translation/rotation injuries of the lower cervical spine are unstable and surgical treatment must be indicated. It is our impression that posterior approach allows a better reduction and stabilization of this injuries and should be used initially without even trying a preoperative traction.

Surgical approach in treatment of translation/rotation injuries of the lower cervical spine in 21 patients

Antecedentes El tratamiento de las lesiones del raquis cervical inferior con luxación de una o ambas facetas articulares y desplazamiento de una vértebra sobre la adyacente todavía genera una controversia considerable. Describimos nuestra experiencia en el tratamiento quirúrgico de este tipo de lesiones. Métodos Presentamos 21 casos tratados entre 2003-2010. La situación neurológica fue valorada con la escala de Frankel. El diagnóstico se hizo mediante radiografía (XR), tomografía computerizada (CT) y/o resonancia magnética (MRI). La tracción cervical fue utilizada en 10 casos antes de la cirugía. En su estabilización se utilizó un abordaje posterior y/o anterior. Resultados Los 21 casos presentados fueron tratados mediante cirugía. Se realizó inicialmente un abordaje posterior en 17 casos y se consiguió la reducción completa en 13 de ellos. Los 4 casos donde obtuvimos solo una reducción parcial, la cirugía tuvo que retrasarse por diferentes motivos. El abordaje anterior se utilizó inicialmente en 4 de los 21 casos. En 3 de ellos, la reducción se consiguió previamente mediante tracción y en el cuarto caso se utilizó (..) (AU)

[AIP mutations in familial and sporadic pituitary adenomas: local experience and review of the literature]. / Mutaciones de AIP en adenomas hipofisarios familiares y esporádicos: experiencia local y revisión de la literatura.

Clinically relevant pituitary adenomas occur 3-5 times more frequently than previously thought. The majority are isolated cases, but their presentation can be familial in the setting of known syndromes such as multiple endocrine neoplasia (MEN)-1 and Carney complex. When 2 or more cases of pituitary adenomas occur in the same family in the absence of the above-mentioned syndromes, a diagnosis of FIPA (familial isolated pituitary adenomas) is made, which accounts for 1-2% of all pituitary adenomas. Mutations of the gene AIP (aryl hydrocarbon receptor-interacting protein) may account for 15% of FIPA families (50% of familial acromegaly), and as such the genetic causes continue to be studied. Also mutations in AIP can be detected in sporadic adenomas among young populations (< 30 years of age). We describe the characteristics of FIPA, detailing the study of a spanish family, in this case AIP mutation negative. Also, the reported findings in sporadic adenomas in the young population are detailed, accompanied by the description of a 19- year old patient with an intronic AIP mutation. Multicenter studies have provided understanding of aspects such as mutations in AIP; however, further studies are necessary to identify other genes involved in FIPA and sporadic pituitary adenomas occurring at a young age.

Mutaciones de AIP en adenomas hipofisarios familiares y esporádicos: experiencia local y revisión de la literatura / AIP mutations in familial and sporadic pituitary adenomas: local experience and review of the literature

Los adenomas hipofisarios clínicamente relevantes son 3-5 veces más frecuentes de lo que inicialmente se pensaba. La mayoría son casos esporádicos, pero su presentación puede ser familiar dentro de síndromes conocidos: neoplasia endocrina múltiple (MEN) 1 y complejo de Carney. Cuando se expresan dos o más casos en la misma familia en ausencia de los síndromes anteriores, hablamos de adenomas hipofisarios familiares aislados (familial isolated pituitary adenomas [FIPA]), que suponen un 1-2% de todos los adenomas hipofisarios. Las mutaciones del gen AIP (aryl hydrocarbon receptor-interacting protein) pueden justificar el 15% de las familias con FIPA (el 50% de acromegalia familiar), pero su base genética continúa en estudio. Además, estas mutaciones de AIP se detectan en adenomas aislados en población joven (< 30 años). Se describen las características descritas en los FIPA detallando el estudio de una familia española, en este caso AIP negativa. También se detallan los hallazgos descritos en adenomas esporádicos en población joven con la presentación de una paciente de 19 años acromegálica con mutación de AIP intrónica. Los estudios multicéntricos han permitido conocer aspectos como las mutaciones de AIP, pero continúan siendo necesarios para conocer otros genes involucrados en los FIPA y los adenomas esporádicos que se presentan en edades tempranas (AU)

Clinically relevant pituitary adenomas occur 3-5 times more frequently than previously thought. The majority are isolated cases, but their presentation can be familial in the setting of known syndromes such as multiple endocrine neoplasia (MEN)-1 and Carney complex. When 2 or more cases of pituitary adenomas occur in the same family in the absence of the above-mentioned syndromes, a diagnosis of FIPA (familial isolated pituitary adenomas) is made, which accounts for 1-2% of all pituitary adenomas. Mutations of the gene AIP (aryl hydrocarbon receptor-interacting protein) may account for 15% of FIPA families (50% of familial acromegaly), and as such the genetic causes continue to be studied. Also mutations in AIP can be detected in sporadic adenomas among young populations (< 30 years of age). We describe the characteristics of FIPA, detailing the study of a spanish family, in this case AIP mutation negative. Also, the reported findings in sporadic adenomas in the young population are detailed, accompanied by the description of a 19-year old patient with an intronic AIP mutation. Multicenter studies have provided understanding of aspects such as mutations in AIP; however, further studies are necessary to identify other genes involved in FIPA and sporadic pituitary adenomas occurring at a young age (AU)