Mandibular metastasis as a presenting feature of a clival chordoma

Chordomas are rare tumors which arise from the embryological remnants of the notochord. These tumors can potentially arise from any region within the craniospinal axis and often clinically present as a diagnostic challenge. Chordomas are rare in patients younger than 40 years of age. The most common primary cancers that metastasize to the jaw bones are the ones originating from the breast, lung, kidney adrenal, colo-rectum, or prostate. Mandibular metastasis from a primary chordoma is an extremely rare occurrence with only five prior reports, three originating from primaries in the sacrococcygeal region, one from a lumbar spine primary and the other from a primary arising from the spheno-occipital region. A literature review did not reveal any prior reports of mandibular metastasis at presentation from a clival chordoma. We possibly report the first case of such an unusual clinical scenario in a 7-year-old male child and further discuss the evaluation and management of these rare tumors

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.

Clinical experience of resection for nasal skull-base tumors by microsurgical-assisted technique / 临床耳鼻咽喉头颈外科杂志

OBJECTIVES@#To investigate the application of the microsurgical treatment in nasal skull-base tumors resection.@*METHODS@#In a retrospective study, totally 15 cases with tumors in the nasal skull-base received microsurgical-assisted treatment in our department from February 2012 to June 2017 were analysed. Lateral rhinotomy approach was carried out in 11 patients and posterior wall of the maxillary sinus approach in 4 patients.@*RESULTS@#Tumors of all cases were completely resected under the microscope. Postoperative bleeding, cerebrospinal fluid leakage, infection and meningo-encephalocele did not occur in this series. The postoperative follow-up time were 6 months to 5 years. One case lost follow-up, seven cases were survivor of tumor-free. Seven cases had recurrence or metastasis, with one case died and other six alive with tumor.@*CONCLUSIONS@#Microsurgical-assisted resection for nasal skull-base tumors can obtain clear vision, with high surgical precision and security.

Endoscopic Endonasal Transsphenoidal Pituitary Tumors Approach: Nuances of Neurosurgical Technique / Abordagem endoscópica endonasal transesfenoidal para tumores hipofisários: detalhes da técnica neurocirúrgica

Pituitary tumors are responsible for 7 to 17% of all intracranial lesions. Over the past decade, advances in endoscopic microsurgical techniques have resulted in an increasingly aggressive endonasal approach to tumors of the midline skull base. We present our series emphasizing technical nuances of endoscopic endonasal transsphenoidal approach to treat pituitary adenomas.

Tumores hipofisários são responsáveis por 7 a 17% de todas as lesões intracranianas. Durante a última década, avanços nas técnicas microcirúrgicas endoscópicas resultaram no aumento de acessos endonasais agressivos para tumores de base de crânio de linha média. Apresentamos nossa série de casos, com ênfase nos detalhes técnicos do acesso endoscópico endonasal transesfenoidal para o tratamento de tumores da hipófise.

The Jugular Foramen Schwannomas: Review of the Large Surgical Series

OBJECTIVE: Jugular foramen schwannomas are uncommon pathological conditions. This article is constituted for screening these tumors in a wide perspective. MATERIALS: One-hundred-and-ninty-nine patients published in 19 articles between 1984 to 2007 years was collected from Medline/Index Medicus. RESULTS: The series consist of 83 male and 98 female. The mean age of 199 operated patients was 40.4 years. The lesion located on the right side in 32 patients and on the left side in 60 patients. The most common presenting clinical symptoms were hearing loss, tinnitus, disphagia, ataxia, and hoarseness. Complete tumor removal was achieved in 159 patients. In fourteen patients tumor reappeared unexpectedly. The tumor was thought to originate from the glossopharyngeal nerve in forty seven cases; vagal nerve in twenty six cases; and cranial accessory nerve in eleven cases. The most common postoperative complications were lower cranial nerve palsy and facial nerve palsy. Cerebrospinal fluid leakage, meningitis, aspiration pneumonia and mastoiditis were seen as other complications. CONCLUSION: This review shows that jugular foramen schwannomas still have prominently high morbidity and those complications caused by postoperative lower cranial nerve injury are life threat.

Anterior Approaches to Midline Skull Base Tumors

Among various approaches to midline skull base tumors, anterior approaches can provide excellent visualization of the lesion. Since June 1989, 12 anterior procedures have been carried out on 9 consecutive patients presenting with midline skull base tumors(four transsphenodal approches, three Le Fort I osteotomies. Two lateral rhinotomies, two facial translocation approaches, and one craniofacial approach). These anterior procedures allowed good access to the lesions. Wound healing was rapid, with little discomfort to the patients. Cosmetic results were also excellent, and there were no significant problems related to malocclusion in the cases of Le Fort I osteotomy. Patients who underwent facial translocation approach developed nasolacrimal duct obstruction and small area hypesthesia on the cheek. Postoperatively, two patients died from tumor progression and meningitis secondary to CSF leakage, respectively. Although the number of cases and follow-up period are limited in our series, we think that anterior approaches may be useful in the surgical treatment to midline skull base tumors.

Improved Access to Skull Base Tumors by Resection of the Zygoma

Improved access to lesions at the medial end of the sphenoid ridge, in the cavernous sinus, or in the interpeduncular cistern after mobilization of the zygoma has been a subject of growing interest in recent years. This study described out experience with 23 patients who underwent the zygomatic osteotomy for skull base tumors in the past 6 years. The follow-up period ranged from 5 months to 49 months. The patient' age range was 11 to 75 years, with an average age of 45 years. This zygomatic osteotomy was used from eleven patients with medial sphenoid ridge lesions, six with lesions arising in or involving the cavernous sinus, three with clival lesions, two with sellar/parasellar lesions, and one with temporal lobe lesions. The histology of these patients showed fourteen meningiomas, two pituitary adenomas, two chordomas, one neurinoma, one chondrosarcoma, one osteochondroma, one malignant lymphoma, and one dermoid cyst. Surgical treatment consisted of total removal in ten patients, subtotal removal in twelve, and partial removal in one. Instances of morbidities associated with basic lesions included cranial nerve injury in five patients, hemorrhage/infarction in three, hemiparesis in two, and transient aphasia in one. There were no significant problems related to zygomatic osteotomy. One patient who underwent zygomatic osteotomy for medial sphenoid ridge meingioma developed a frontalis nerve injury. No patient experienced a detachment of zygomatic arch in our series. Postoperatively, one patient with parasellar malignant lymphoma died 14 months after surgery from tumor progression. Our cases treated via this zygomatic osteotomy are as yet insufficient to determine whether the method offers definite adventages, in terms of patient mortality and morbidity, over conventional operative approaches, but we suggest that this procedure has some advantages such as minimal brain retraction, exposure of lesion in shortest distance, multidirectional viewing of the lesions, and can serve as an alternative approach to a usual pterional approach when cranial base pathologies are large or complex.