Jugular Foramen Paragangliomas.

Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

Microsurgical Resection of Giant Petroclival Meningioma via the Posterior Petrosal Approach: 2-Dimensional Operative Video.

Petroclival meningiomas are challenging deep-seated lesions related to many critical neurovascular structures of the skull base.1-5 We present the case of a 45-year-old male presenting with a 3-year history of progressive headache associated gradually with multiple cranial nerves deficits and progressive tetraparesis leading to use of a wheelchair (Video 1) Preoperative magnetic resonance imaging demonstrated a mass highly suggestive of a giant left petroclival meningioma. Considering worsening of symptoms and impressive mass effect, microsurgical resection employing the posterior petrosal approach was performed. Mastoidectomy with skeletonization of semicircular canals and a craniotomy approaching both posterior and middle cranial fossae were done. Dural incision at the base of the temporal lobe was communicated to other incision in the presigmoid dura by ligation and sectioning of superior petrosal sinus. Tentorium was cut all the way toward the incisura, with attention to preserve the fifth nerve along its division and fourth nerve in the last cut. After a complete tentorium incision, the presigmoid space enlarged, exposing both supratentorial and infratentorial spaces. The lesion was totally resected employing microsurgical techniques. Postoperative magnetic resonance imaging demonstrated complete tumor resection. The patient experienced improvement of complaints and no new neurologic deficit on follow-up. The posterior petrosal approach gives great exposure and a more lateral angle of attack to the ventral surface of brainstem, allowing in this case to approach the whole tumor attachment. Informed consent was obtained from the patient for the procedure and publication of this operative video. Anatomic images were courtesy of the Rhoton Collection, American Association of Neurological Surgeons/Neurosurgical Research and Education Foundation.

Skull base osteomyelitis mimicking skull base tumor in immunocompetent pediatric patient: case report.

Central skull base osteomyelitis (CSBO) is a rare complication of infection in pediatric patients, especially when there are no comorbidities like immunosuppression or metabolic illness. The diagnosis of CSBO is a challenge in children, and imaging findings can mimic skull base tumor. We describe the clinical history and image diagnosis of a case in a 6-year-old girl with no relevant history who presented an extensive skull base lesion. She underwent tumor resection surgery. The intraoperative finding confirmed clivus osteomyelitis, and the histopathological studies discarded malignancy. After diagnosis, the patient completed 6-week antibiotic treatment with adequate evolution. In conclusion, CSBO should be considered within the differential diagnoses due to the fact that it can mimic skull base lesions and it may present without relevant history.

Spontaneous Regression of a Clival Chordoma: An Unusual Case Report.

INTRODUCTION: Chordoma is a malignant and aggressive tumor originating from remnants of the primitive notochord and usually involving the axial skeleton. Spontaneous regression of clival chordomas was described recently. We present the third case report of spontaneous regression of a clival chordoma and discuss similarities of cases and implications for clinical practice. CASE DESCRIPTION: We present the case of a previously healthy 21-year-old Caucasian woman who presented with progressive holocranial headache for 3 months, which encouraged image investigation. Magnetic resonance imaging (MRI) revealed an osteolytic clival lesion hyperintense in T2 and hypointense in T1 images. After 2 months of initial evaluation and surgical proposal, she repeated MRI to allow use for intraoperative neuronavigation. Surprisingly, there was tumor regression. DISCUSSION: The present reported case is somehow different from previous ones and does not share an underlying inflammatory/immunological recognizable fact, being interpreted by us as a spontaneous partial regression of the tumor. We highlight the need for continuous investigation of chordoma regression to uncover the underlying mechanisms.

Resection of Clival Chordoma Through the Anterior Clivectomy: 2-Dimensional Operative Video.

Clival chordomas are rare malignant behaving tumors that grow, locally invade, metastasize, and seed, and they have a high recurrence rate.1,2 The longest disease control is achieved by radical resection followed by high doses of radiation therapy, commonly proton beam.3 To achieve radical tumor removal, multiple surgical procedures through different approaches might be required.4 Since the chordoma's origin is, and remains, extradural, an extradural approach is preferred, and can lead to intradural extension. Anterior approach is frequently utilized to remove the midline-located tumor and the eroded clivus.5 Several midline approaches were utilized, including the transbasal, transfacial, transcervical, open door, and Lefort's maxillotomies1; however, the same tumor removal can be achieved with a simple extension of the trans-sphenoidal approach, by resecting the anterior maxillary wall, of the contralateral to the lesion preponderant side.5 This approach coupled with the use of neuronavigation on mobile head and endoscopic-assisted technique allowed to achieve a wide and direct exposure, with the ability to resect extra- and intradural tumors.2,5 Lately, the endonasal endoscopic technique became popular as an alternative4; however, we found a great advantage in the ability to combine the stereoscopic microsurgical technique with the endoscopic dissection, in addition to avoiding the extensive nasal dissection and its complications. We present a case of a 63-yr old woman with an upper clivus chordoma compressing the brainstem who underwent a gross total resection by endoscopic-assisted microscopic techniques through an anterior clivectomy approach. Patient consented to the procedure and publication of her images.

Far Lateral Approach for Resection of a Foramen Magnum Meningioma: 3-Dimensional Surgical Video.

We present the case of a meningioma of the foramen magnum, in a patient of age 62 yr, who presented at the time of surgery a hemiparesis on the right side at 2 mo of evolution. The patient gave his informed consent for the publication of the case, and approval was obtained from the research department of the hospital where the procedure was performed. The magnetic resonance imaging (MRI) in the mid-sagittal view shows a meningioma of the foramen magnum, with an extension in the posterior fossa and in the upper part of the cervical canal. In the coronal view and in the axial view, we can identify that although it is a meningioma of the anterior part of the foramen magnum, it is observed that the lesion has a displacement towards the right side. In a cadaveric specimen, we show the normal anatomy and the key landmarks for performing the approach. The patient was treated by a far lateral approach with a partial removal of the condyle. We show the craniectomy and the microsurgical technique for the tumor resection step by step. We paid particular attention in the anatomy surrounding the tumor and the tips and tricks for a safe resection. We reached a total resection with a good outcome; the result of the anatomopathological study confirmed the diagnosis of meningothelial meningioma. The dura was closed in a hermetic manner with a synthetic dura patch; then sealant was placed. The bone defect was corrected by placing a mesh in titanium. Then the flap was closed as usual. The patient has a good evolution with 1 yr of follow-up and without lesion in the control MRI.

[Stereotactic brain radiosurgery in the treatment of skull base tumors.] / Radiocirugía cerebral estereotáctica en el tratamiento de tumores de base de cráneo.

Stereotactic radiosurgery has shown effectiveness in the multimodal treatment of skull base tumors, however it is suggested that in radiobiologically sensitive areas (eg. optic nerves and brainstem) the radiation dose should be reduced due to radiotoxic effects. To avoid the consequent therapeutic ineffectiveness, a recently implemented alternative is multisession radiosurgery (RCH). We retrospectively analyzed a series of patients with skull base tumors, in order to evaluate the treatment with RCH through tumor control in the post-treatment images. Age, sex, tumor histology, tumor volume, radiation protocol, pre-treatment Karnofsky performance status (KPS) previous neurosurgery and radiant treatment were also evaluated. Eighty-four patients were treated between January 2009 and January 2017. The median age was 51.5 years; females: 53.6%. There was a 92.7% non-progression after treatment, with a median follow-up time of 36 months. Treated tumors were mainly pituitary adenomas, acoustic schwannomas, and skull base meningiomas. Most of the patients received a 5-day fractionated treatment scheme, with a total dose of 25 Gy. No clinically manifest late radiotoxicity was observed. In the multivariate analysis, a high pre-treatment KPS was significantly associated with tumor non-progression. In our series, the high incidence of tumor non-progression would indicate that treatment with RCH could be a therapeutic option in some cases of skull-base tumors, mainly recurrences or tumor residuals of pituitary adenomas, neurinomas and meningiomas.

La radiocirugía estereotáctica ha demostrado efectividad en el tratamiento multimodal de los tumores de base de cráneo, de todas maneras se sugiere que en áreas radiobiológicamente sensibles (ej. nervios ópticos y tronco cerebral) la dosis radiante debería reducirse por los efectos radiotóxicos. Para evitar la ineficacia terapéutica, una alternativa recientemente implementada es la radiocirugía multisesión (RCH). Se analizó en forma retrospectiva una serie de tumores de base de cráneo, con el fin de evaluar el tratamiento con RCH a través del control tumoral en las imágenes post-tratamiento. Se evaluó también edad, sexo, histología tumoral, volumen tumoral, protocolo radiante, Karnofsky performance status (KPS) pre-tratamiento y neurocirugía y tratamiento radiante previos. Fueron tratados 84 pacientes entre enero 2009 y enero 2017. La mediana de edad fue 51.5 años; sexo femenino: 53.6%. Hubo un 92.7% de no-progresión luego del tratamiento, con una mediana de tiempo de seguimiento de 36 meses. Los tumores tratados fueron principalmente adenomas hipofisarios, neurinomas del acústico, y meningiomas de base de cráneo. La mayoría de los pacientes recibió un esquema de tratamiento fraccionado de 5 días, con una dosis total de 25 Gy. No se observó radiotoxicidad tardía clínicamente manifiesta. En el análisis multivariado, un KPS alto pre-tratamiento fue significativamente asociado a la no-progresión tumoral. En esta serie, la alta incidencia de no-progresión tumoral indicaría que el tratamiento con RCH podría ser una opción terapéutica en algunos casos de tumores de base de cráneo, principalmente recurrencias o remanencias tumorales de adenomas hipofisarios, neurinomas y meningiomas.

Radiocirugía cerebral estereotáctica en el tratamiento de tumores de base de cráneo / Stereotactic brain radiosurgery in the treatment of skull base tumors

La radiocirugía estereotáctica ha demostrado efectividad en el tratamiento multimodal de los tumores de base de cráneo, de todas maneras se sugiere que en áreas radiobioló;gicamente sensibles (ej. nervios ó;pticos y tronco cerebral) la dosis radiante debería reducirse por los efectos radiotó;xicos. Para evitar la ineficacia terapó;©utica, una alternativa recientemente implementada es la radiocirugía multisesió;n (RCH). Se analizó; en forma retrospectiva una serie de tumores de base de cráneo, con el fin de evaluar el tratamiento con RCH a travó;©s del control tumoral en las imágenes post-tratamiento. Se evaluó; tambín edad, sexo, histología tumoral, volumen tumoral, protocolo radiante, Karnofsky performance status (KPS) pre-tratamiento y neurocirugía y tratamiento radiante previos. Fueron tratados 84 pacientes entre enero 2009 y enero 2017. La mediana de edad fue 51.5 aó;±os; sexo femenino: 53.6%. Hubo un 92.7% de no-progresió;n luego del tratamiento, con una mediana de tiempo de seguimiento de 36 meses. Los tumores tratados fueron principalmente adenomas hipofisarios, neurinomas del acó;ºstico, y meningiomas de base de cráneo. La mayoría de los pacientes recibió; un esquema de tratamiento fraccionado de 5 días, con una dosis total de 25 Gy. No se observó; radiotoxicidad tardía clínicamente manifiesta. En el análisis multivariado, un KPS alto pre-tratamiento fue significativamente asociado a la no-progresió;n tumoral. En esta serie, la alta incidencia de no-progresió;n tumoral indicaría que el tratamiento con RCH podría ser una opció;n terapó;©utica en algunos casos de tumores de base de cráneo, principalmente recurrencias o remanencias tumorales de adenomas hipofisarios, neurinomas y meningiomas.

Stereotactic radiosurgery has shown effectiveness in the multimodal treatment of skull base tumors, however it is suggested that in radiobiologically sensitive areas (eg. optic nerves and brainstem) the radiation dose should be reduced due to radiotoxic effects. To avoid the consequent therapeutic ineffectiveness, a recently implemented alternative is multisession radiosurgery (RCH). We retrospectively analyzed a series of patients with skull base tumors, in order to evaluate the treatment with RCH through tumor control in the post-treatment images. Age, sex, tumor histology, tumor volume, radiation protocol, pre-treatment Karnofsky performance status (KPS) previous neurosurgery and radiant treatment were also evaluated. Eighty-four patients were treated between January 2009 and January 2017. The median age was 51.5 years; females: 53.6%. There was a 92.7% non-progression after treatment, with a median follow-up time of 36 months. Treated tumors were mainly pituitary adenomas, acoustic schwannomas, and skull base meningiomas. Most of the patients received a 5-day fractionated treatment scheme, with a total dose of 25 Gy. No clinically manifest late radiotoxicity was observed. In the multivariate analysis, a high pre-treatment KPS was significantly associated with tumor non-progression. In our series, the high incidence of tumor non-progression would indicate that treatment with RCH could be a therapeutic option in some cases of skull-base tumors, mainly recurrences or tumor residuals of pituitary adenomas, neurinomas and meningiomas.

Determination of Normal Values of the Basal Angle in the Era of Magnetic Resonance Imaging.

BACKGROUND: The flexion of the skull base (basal angle [BA]) is the inclination between the anterior fossae and the basilar ramp of the occipital and sphenoid bones. An increased BA, termed platybasia, is usually associated with basilar invagination. BA reference values in the magnetic resonance imaging (MRI) era and the diagnosis of platybasia are of clinical importance. The transnasal approach has been the surgical technique of choice to remove the odontoid process in cases of ventral brainstem compression in patients with platybasia. The knowledge of normal BA values has been influenced by technological image acquisitions. The aim of this study was to determine the normal BA values in normal subjects in the MRI era. METHODS: For the determination of normal BA values, the literature reporting these values in normal individuals was reviewed and a meta-analysis of pertinent studies was performed. RESULTS: A total of 8 articles were included in this study, evaluating a total of 667 individuals. A summary measure of the results was obtained. The data obtained in this analysis provided a final basal angle value of 116.5 degrees (95% confidence interval, 104.39-128.7). The normal basal angle ranges between 104 and 129 degrees. CONCLUSION: Platybasia can be defined as a value >129 from the basal angle.

Atypical Intracranial Compound Simil Odontoma.

Odontoma is the most common mixed epithelial and mesenchymal tumour-like malformation which affects maxillary bones. Clinically, Odontomas present as lesions of benign behavior, causing little or no deformity, are asymptomatic, with self-limited growth and usually detected on routine radiographs. Radiographically, they are radiopaque lesions, well-demarcated surrounded by a thin soft tissue capsule. Compound odontomas consist of numerous tooth-like structures, whereas in complex odontomas it consists of a disorganized mass of calcified tissue. The aim of this article was to report an atypical case of intracranial compound odontoma, in the middle cranial fossa, reaching the sphenoidal sinus, which has been followed for 10 years.

Estesioneuroblastoma Olfatorio: Presentacion De Un Caso / Oligatory Esthesioneuroblastoma: Presentation of a Case

Se presenta un caso de una paciente con pérdida de peso, congestión nasal epistaxis, aumento de volumen en cuello con disfagia a sólidos y líquidos de 1 mes de evolución. La tomografía de cuello muestra una masa de tejidos blandos en la base de cuello con erosión del esfenoides con extensión a la fosa craneal media, con erosión del clivus, el esfenoides y la si­lla turca. El diagnostico histopatológico es un estesioneuroblastoma.

We present a case of a patient with weight loss, nasal congestion, epistaxis, increase neck volu­ me with dysphagia to solids and liquids of 1 month of evolution. The neck tomography shows a soft tissue mass at the base of the neck with erosion of the sphe­ noid with extension to the middle cranial fossa, with erosion of the clivus, the sphenoid and the sella turcica. The histopathological diagnosis is an esthesioneuroblastoma.

Three-Dimensional Volume Imaging to Increase the Accuracy of Surgical Management in a Case of Recurrent Chordoma of the Clivus.

BACKGROUND The clivus is a depression in the anterior occipital bone of the skull base, posterior to the dorsum sellae, at the junction with the sphenoid bone. Chordoma is a rare tumor arising from embryonic remnants of the notochord and can be locally aggressive with a tendency to recur. The optimal management of this rare tumor remains controversial. A report of a case of recurrent chordoma of the clivus is presented to illustrate the value of volumetric three-dimensional (3-D) reconstruction with computed tomography (CT) and magnetic resonance imaging (MRI) to determine optimal surgical management. CASE REPORT A 53-year-old man presented with pain in the right orbital cavity, right proptosis, swelling of the right cheek, and bilateral loss of vision. He also had adrenal insufficiency. CT and contrast-enhanced (gadolinium) T1-weighted MRI with multiplanar acquisition were performed with volumetric 3-D reconstruction of the tumor, to increase the chances of treatment success. Surgical resection was performed to remove the tumor and reduce the risk of recurrence. Histology of the tumor was consistent with chordoma, supported by positive immunohistochemical staining for S-100 and epithelial membrane antigen (EMA). CONCLUSIONS This report highlighted the value of 3-D volume imaging in the diagnosis and treatment planning in a rare case of recurrent chordoma of the clivus. Analysis of tumor volume may be an indicator of the efficacy of surgery, complementing the Response Evaluation Criteria In Solid Tumors (RECIST) system and as a valuable tool to predict treatment outcome.

Olfactory Groove Schwannoma ­ A Case Report and Literature Review

Olfactory groove schwannomas (OGSs) are rare benign tumors of the anterior skull base region. Considering the lack of Schwann cells in the optic and olfactory nerves, their origin remains enigmatic. Despite the precursor cell, total resection of the lesion is curative, as long as the histopathological features of the tumor are compatible with schwannoma. We report the case of a 32-year-old woman, addicted to crack, who was brought to the hospital presenting with cognitive dysfunction after being physically assaulted, whose neuroimaging revealed a large extra-axial mass in the subfrontal sagittal region. The presentation, immunohistochemical markers and histogenesis are discussed in the present study, along with a literature review.

Biopsia endoscópica de osteocondroma de fosa infratemporal / Endoscopic biopsy of infratemporal fossa osteochondroma

Presentamos el caso de una mujer de 13 años con un gran tumor de características óseas en la fosa infratemporal derecha, el cual fue biopsiado mediante un abordaje endoscópico transeptal transpterigoídeo. La biospia mostró un osteocondroma. Describimos el caso y discutimos sus aspectos relevantes.

We report the case of a 13-year-old woman with a large tumor with osseous appearance in her right infratemporal fossa, which was biopsied through an endoscopic transpterygoid approach. The biopsy showed an osteocondroma. We described the case and discuss its relevant aspects.