Cordoma Sacral: Relato de uma Rara Neoplasia Maligna / Sacral Chordoma: Report of a Rare Malignant Neoplasm / Cordoma Sacral: Relato de uma Rara Neoplasia Maligna

Introdução: O cordoma é um tipo de sarcoma cuja malignidade óssea primária se origina da notocorda e se localiza no eixo espinhal entre o clivus e o sacro. A primeira descrição dessa patologia foi em 1857. Na epidemiologia da doença, são mais afetados pacientes entre 40 e 60 anos, sendo o principal sítio de acometimento a região sacrococcígea. O quadro clínico é variável conforme o local acometido com sintomas geralmente inespecíficos, gerando atrasos no diagnóstico feito por biópsia. Entre as opções de tratamento, o principal método é a ressecção cirúrgica com margens, que pode ser associada à radioterapia ou à radiocirurgia quando necessário; os sítios de metástases mais comuns são pulmões, ossos, fígado e linfonodos locais. Relato do caso: Paciente, sexo feminino, 62 anos, iniciou com quadro de dor em região coccígea com piora ao sentar-se e surgimento de lesão nodular com crescimento progressivo recebendo diagnóstico de cordoma, após biópsia da lesão, depois de três anos. Em razão da lesão extensa, optou-se inicialmente por tratamento com quimio e radioterapia para citorredução. Pela pouca responsividade, foi submetida ao tratamento de sacralectomia com sucesso, porém apresentou como complicação deiscência de ferida operatória e necessidade de reabordagem, desde então sem recorrência no seguimento clínico. Conclusão: Assim, evidencia-se a necessidade de novas pesquisas sobre o cordoma, um tumor raro e de baixa responsividade aos tratamentos não cirúrgicos, visando a melhorar a terapêutica quimioterápica dessa neoplasia potencialmente deformante.

Introduction: Chordoma is a type of sarcoma, a primary bone malignancy that originates from the notochord and is located on the spinal axis between the clivus and the sacrum. The first description of this pathology occurred in 1857. Patients between 40 and 60 years old are the most affected according to the disease's epidemiology, the main site involved is the sacral/coccygeal region. The clinical condition is variable depending on the site affected, with generally nonspecific symptoms, delaying the diagnosis made by biopsy. Among the treatment options, surgical resection with margins is currently the main method, and may be associated with radiotherapy or radiosurgery when necessary; the most common metastatic sites are lungs, bones, liver and local lymph nodes. Case report: A 62-year-old female patient had pain in the coccygeal region, worsening while sitting and the appearance of a nodular lesion with progressive growth, diagnosed as a chordoma three years later, after biopsy of the lesion. Due to the extensive lesion, initially she was submitted to chemotherapy and radiotherapy for cytoreduction, but because of the poor response, she was successfully submitted to sacralectomy, however, dehiscence of the surgical wound was detected, and the patient underwent a new approach; since then, no recurrence in the clinical follow-up. Conclusion: Apparently, it is clear the necessity for further investigations on chordoma, a rare tumor with poor response to non-surgical treatments, in order to improve the chemotherapy for this potentially deforming neoplasm.

Introducción: El cordoma es un tipo de sarcoma, una malignidad ósea primaria que se origina en la notocorda y se localiza en el eje espinal entre el clivus y el sacro. La primera descripción de esta patología fue en 1857. En la epidemiología de la enfermedad, los pacientes entre 40 y 60 años son los más afectados, siendo el principal sitio de afectación la región sacrocoxígea. El cuadro clínico es variable según el sitio afectado, con síntomas generalmente inespecíficos, lo que provoca retrasos en el diagnóstico realizado mediante biopsia. Entre las opciones de tratamiento, la resección quirúrgica con márgenes es actualmente el principal método, pudiendo asociarse a radioterapia o radiocirugía cuando sea necesario; los sitios más comunes de metástasis son los pulmones, los huesos, el hígado y los ganglios linfáticos locales. Informe del caso: Paciente, sexo femenino, de 62 años inició con dolor en la región coccígea, empeorando al sentarse y aparición de una lesión nodular con crecimiento progresivo, recibiendo diagnóstico de cordoma, luego de biopsia de la lesión, después de tres años. Debido a la extensión de la lesión optó inicialmente por tratamiento con quimio y radioterapia para citorreducción, por la poca reactividad fue sometida con éxito al tratamiento de sacralectomía, pero presentó como complicación dehiscencia de la herida quirúrgica y necesidad de reabordaje. Desde entonces sin recurrencia en el seguimiento clínico. Conclusión: Por lo tanto, es evidente la necesidad de seguir investigando sobre el cordoma, un tumor poco frecuente con escasa respuesta a los tratamientos no quirúrgicos, con el fin de mejorar la terapia de quimioterapia para esa neoplasia potencialmente deformante.

Cordoma sacral metastático / Metastatic sacral chordoma

Os cordomas sacrais (CS) são tumores ósseos malignos primários da coluna vertebral de ocorrência rara, com incidência entre 0,000005-0,000027%. O objetivo deste estudo é relatar um caso de CS metastático. Homem de 41 anos, sem comorbidades, chega ao serviço de referência apresentando lesão sacral. Ressonância magnética mostrou tratar-se de tumor com 9,3 cm sugestivo de mieloma ou cordoma. Realizou-se biópsia e histopatológico, confirmando o diagnóstico de CS. O paciente submeteu-se à excisão cirúrgica do tumor. Seis meses após a cirurgia, evoluiu com recidiva e implantes metastáticos em coluna vertebral, partes moles da parede torácica, fígado e espa-ço pleural, evoluindo com paraplegia. Não havia indicação de radioterapia e/ou quimioterapia adjuvante. Não havia também possibilidade de liberação de imatinibe pelo Sistema Único de Saúde. Em cerca de 28 meses de seguimento clínico mensal, o paciente foi a óbito. O caso apresentado mostrou um CS sem sucesso cirúrgico, o que é associa-do a pior prognóstico. O paciente apresentou disseminação sistêmica do tumor e paraplegia poucos meses após a cirurgia, indo a óbito em 28 meses de seguimento. (AU)

Sacral chordomas (SC) are rare primary malignant bone tumors of the vertebral column, with an incidence between 0.000005-0.000027%. This study aims to describe a case of metastatic SC. A 42-year-old man without comorbid conditions, arrived at the referral center, presenting with a sacral lesion. MRI showed a tumor measuring 9.3 cm that was suggestive of myeloma or chordoma. A biopsy with histopathology study was performed, confirming the diagnosis of SC. The patient underwent surgical tumor excision. Six months after surgery, the tumor recurred with metastatic vertebral column implants, soft tissues of the chest wall, liver, and pleural space, and the patient developed paraplegia. There was no indication of adjuvant radiotherapy and/or chemotherapy. There was also no possibility that the Unified Health System would approve imatinib. At about 28 months of monthly clinical follow-up, the patient died. The case presented showed unsuccessful SC surgery, which is associated with a worse prognosis. The patient had systemic tumor dissemination and paraplegia a few months after surgery, dying at 28 months of follow-up. (AU)

Cordomas cervicales. Reporte de casos / Cervical chordomas. Case report

Introducción La detección temprana del cordoma constituye un desafío médico dada su baja frecuencia, calculada entre 2-4% de todos los tumores primarios del hueso, requiriendo un alto índice de sospecha clínica. El diagnóstico tardío incrementa la morbimortalidad y compromete la sobrevida del paciente, por lo que el conocimiento actualizado sobre su etiopatogenia, clínica y tratamiento es de suma importancia para el cirujano espinal, quien forma parte esencial del grupo interdisciplinario terapéutico. Reporte de casos Reportamos dos casos de cordomas cervicales, localmente agresivos sin extensión sistémica, el primero de ellos ubicado en la columna cervical y el segundo en la unión craneocervical. Ambos con diferentes formas de presentación, síntomas, tiempo de evolución (5 años y 7 meses respectivamente), infiltración tumoral y abordaje quirúrgico, pero similar tratamiento oncológico. El objetivo del manuscrito es demostrar la variedad de presentación cervical de la entidad y las diferentes formas de abordarla. Se incluye una revisión actualizada de la literatura donde se evidencia la importancia de la inmunoterapia como nuevo horizonte terapéutico, y se reafirma la resección quirúrgica como base del mismo. Discusión Se presentan los resultados imagenológicos de descompresión medular, resección ósea y de partes blandas, los tipos de fijación espinal y la evolución clínica, sin recidiva local a los 2 años y a los 12 meses respectivamente en cada caso, luego del manejo quirúrgico y la radioterapia adyuvante.

Background Early detection of chordoma is a medical challenge given its low frequency, a high index of clinical suspicion is required for the diagnosis. The late diagnosis increases morbidity and mortality and compromises the survival of the patient. Knowledge about its etiology, clinical manifestations and treatment is of utmost importance for the spinal surgeon as well as for the therapeutic group. The aim of the report is to demonstrate the variety of cervical presentation of the entity and the different ways of approaching it. Case Report We report two cases of locally aggressive cervical chordomas without systemic extension, the first one located in the cervical spine and the second in the craniocervical junction. Both with different forms of presentation, symptoms, evolution time (5 years and 7 months respectively), tumor infiltration and surgical approach, but similar oncological treatment. Discussion The imaging results of medullar decompression, bone and soft tissue resection, types of spinal fixation and clinical evolution were presented. No local recurrence at 2 years and 12 months respectively after surgical management and adjuvant radiation therapy were found.

Metastatic poorly differentiated chordoma: the eyes do not see what the mind does not know

Chordoma is a rare tumor. It has unique clinical, pathological and immunohistochemical characteristics. Accurate diagnosis is essential as the tumor shows an aggressive clinical course and requires a multimodal therapeutic approach. A case with wide spread distant metastatic disease that was initially thought to represent metastatic thyroid carcinoma is presented. Appropriate clincopathologic correlation and the histologic findings raised the possibility of poorly differentiated chordoma. The diagnosis was confirmed by immunohistochemistry for INI-1 and Brachyury. The approach to the diagnosis emphasizing the clinical and pathologic findings of this case is discussed and reviewed in the context of the published literature.

Reto neuroquirúrgico actual del cordoma cervical / Current neurosurgical challenge of the cervical chordoma

El cordoma es un tumor osteocartilaginoso raro, de lento crecimiento, con una tasa de incidencia global de 8,4 casos por cada 10 millones de habitantes. Comúnmente aparece en la quinta y sexta década de la vida, predomina en el sexo masculino. Se presenta un paciente masculino de 47 años de edad, con cervicobraquialgia, cuadriparesia y masa tumoral palpable en región anterolateral del cuello. El estudio de resonancia magnética demostró la presencia de una lesión retrofraríngea con destrucción vertebral y compresión extradural. Se decidió resección quirúrgica de la lesión. El diagnóstico histopatológico por inmunohistoquímica arrojó como resultado, un cordoma(AU)

Chordoma is a rare, slow-growing osteocartilaginous tumor with an overall incidence rate of 8.4 cases per 10 million inhabitants. Commonly appears in the fifth and sixth decade of life, predominates in the male sex. We present a 47-year-old male patient with cervicobrachialgia, quadriparesis and palpable tumor mass in the anterolateral region of the neck. The magnetic resonance study showed the presence of a retropharyngeal lesion with vertebral destruction and extradural compression. Surgical resection of the lesion was decided. The histopathological diagnosis by immunohistochemistry resulted in a chordoma(AU)

Sacrectomía parcial por abordaje posterior único / Partial sacrectomy by single posterior approach

Introducción: Los tumores del sacro representan <7% de los tumores espinales, prevalecen los tumores secundarios por mieloma múltiple o carcinomas de próstata, mama, pulmón o colón. El cordoma es el tumor maligno primario más frecuente y el tumor de células gigantes es la lesión benigna más común. Por su evolución, compromiso de estructuras extraóseas y la escasa respuesta a los tratamientos coadyuvantes, la cirugía es el tratamiento más utilizado, la vía de abordaje y la necesidad de instrumentación dependerán del tumor por tratar. Los objetivos de este trabajo son: evaluar el uso de la resección parcial del sacro, analizar la técnica quirúrgica y reconocer las complicaciones. Materiales y Métodos: Cuatro pacientes con diagnóstico de tumor sacro ubicado por debajo de S1, que consultan por dolor y cuyas imágenes confirman la lesión. A todos se les realiza una resección en bloque por vía posterior preservando S1. Se describe la técnica. Resultados: Los estudios anatomopatológicos revelaron: un cordoma, un tumor maligno de vaina nerviosa, un condrosarcoma y una metástasis de carcinoma prostático. Se preservó la función de S1 en todos los pacientes; uno tiene disfunción vesical permanente. Se observaron una dehiscencia de la herida, una infección y una fístula de líquido cefalorraquídeo. Todos permanecen sin la enfermedad tras un seguimiento de entre 6 y 24 meses. Conclusiones: La resección parcial del sacro por abordaje posterior único se puede indicar cuando la lesión compromete desde S2 hacia distal y no hay compromiso sacroilíaco. La preservación de raíces es de vital importancia para garantizar mejores resultados posoperatorios y una menor tasa de infección. Nivel de Evidencia: IV

Introduction: Tumors of the sacrum represent less than 7% of spinal tumors. Secondary tumors due to multiple myeloma or prostate, breast, lung and colon carcinomas predominate. Chordoma is the most frequent primary malignant tumor and giant cell tumor is the most common benign lesion. For its evolution, involvement of extra bone structures and poor response to the adyuvant therapy, surgery is the most commonly used treatment, the approach and the need for instrumentation depend on tumor to treat. The objectives of this study are to evaluate the use of partial resection of sacrum, analyze the surgical technique and assess complications. Methods: Four patients with diagnosis of sacral tumor below S1, who presented with pain and images confirming the injury. All underwent an en bloc resection by posterior via preserving S1. The technique is described. Results: The pathological results were: a chordoma, a malignant nerve sheath tumor, a chondrosarcoma and a metastasis of prostatic carcinoma. S1 function was preserved in all patients; one has permanent bladder dysfunction. Wound dehiscence, infection and cerebrospinal fluid fistula were detected. Patients are free of disease after a follow-up of 6-24 months. Conclusions: Partial resection of the sacrum using a unique posterior approach may be indicated when the injury involves from S2 distally and no sacroiliac involvement is observed. Root preservation is vital to guarantee the best postoperative results and a lower rate of infection. Level of Evidence: IV

Cordoma en la articulación facetaria de C4: reporte de caso / Chordoma in C4 Facet Joint: A Case Report

Los cordomas son tumores óseos primarios, poco frecuentes, derivados de remanentes no diferenciados de la notocorda. Por su origen histológico, suelen ubicarse en la línea media del esqueleto axial, y los lugares de presentación más frecuentes son la base del cráneo y la columna. Se caracterizan por presentar un crecimiento lento, por lo que tienden a ser clínicamente silenciosos hasta alcanzar tamaños que causan manifestaciones que varían según el sitio de presentación; sin embargo, tienen alta agresividad y recurrencia local. El tratamiento es quirúrgico e, idealmente, se busca una resección completa de la lesión. El artículo presenta el caso de un hombre de 20 años de edad, quien desarrolló un cordoma en la articulación facetaría superior izquierda de C4, que es una localización rara.

Chordoma are rare primar? bone tumours derived from non'difieren tiated remains of the notochord. Due to their histological origina, the most common site of presentation is on the mid'line of the axial skeleton, with a distribution that is most frequent on the sacral bone, skull base and mobile spine. These tumours have a slow growth rate, which means that symptoms occur when the size of the mass causes different manifestations according to its site of presentation. Howeveti they have aggressive behaviour with high rates of local recurrence. Ideal treatment is based on surgical block removal if possible. This article presents the case of a 20' year'old male patient with diagnosis of a chordoma on the leít superior facetar? articulation of C4.

Cordoma sacrococcígeo en un paciente de mediana edad / Sacrococcigeal chordoma in a middle age patient

Se presenta el caso clínico de un hombre de 48 años de edad, quien fuera ingresado en el Hospital Clinicoquirúrgico Docente "Dr. Joaquín Castillo Duany" de Santiago de Cuba en el 2007, por presentar dificultades para defecar. Según valoración clínico-radiológica, se diagnosticó un cordoma sacrococcígeo y fue egresado debido al estadio del tumor, con indicación de seguimiento en consulta externa. En el mes de julio de ese mismo año se le hospitalizó en el Instituto Nacional de Oncología y Radiología, donde fue intervenido quirúrgicamente. Un año más tarde fue ingresado en el Hospital General Docente "Dr. Juan Bruno Zayas Alfonso", con signos de recidiva tumoral de gran extensión. Falleció 6 meses después.

The case report of a 48 year-old man who was admitted in "Dr. Joaquín Castillo Duany" Teaching Clinical Surgical Hospital from Santiago de Cuba in the 2007 is presented, due to difficulties for defecating. According to a clinical-radiological evaluation, a sacrococcigeal chordoma was diagnosed and he was discharged due to the tumor stage, with the indication of follow up through out patient department. In the month of July of that same year, he was hospitalized in the National Institute of Oncology and Radiology, where he was surgically treated. A year later, he was admitted in "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital, with signs of tumor relapse of great extension. He died 6 months later.

Natural history and surgical treatment of chordoma: a retrospective cohort study / História natural e tratamento cirúrgico de cordoma: um estudo retrospectivo de coorte

CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition. DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil. METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test. RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5%) were located in the sacrum, eleven (26.2%) in the skull base and six (14.3%) in the mobile spine. Surgery was performed on 28 patients (66.7%). The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS) was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins). In the inoperable group, OS was 37.7% at 24 months and 0% at five years. CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival. .

CONTEXTO E OBJETIVO: Cordoma é um tumor raro e com alto risco de recidiva locorregional. O objetivo deste estudo foi analisar os resultados a longo prazo do tratamento dessa doença. TIPO DE ESTUDO E LOCAL: Estudo de coorte realizado em um único hospital em São Paulo, Brasil. MÉTODOS: Estudo de coorte retrospectivo com 42 pacientes com cordoma tratados de 1980 e 2006 no Hospital A. C. Camargo. Os prontuários foram revistos e foi realizada a análise descritiva das variáveis clínicas e patológicas. As curvas de sobrevida foram estimadas pelo método de Kaplan-Meier e a comparação entre elas, pelo teste de log-rank. RESULTADOS: Dezenove pacientes eram homens e 23, mulheres. Vinte e cinco tumores (59,5%) estavam localizados no sacro, 11 (26,2%) na base do crânio e 6 (14,3%), na coluna móvel. A cirurgia foi realizada em 28 pacientes (66,7%). A ressecção foi considerada como tendo margens negativas em 14 casos e margens comprometidas em 14 pacientes. A sobrevida global (SG) em 5 anos foi de 45,4%. Para os pacientes cirúrgicos, a SG em 5 anos foi de 64,3% (82,2% para as margens negativas e 51,9% de margens positivas). No grupo inoperável, a SG em 24 meses foi de 37,7% e 0% em 5 anos. CONCLUSÃO: A ressecção completa está relacionada com o controle local e, definitivamente, tem impacto positivo na sobrevida a longo prazo. .

Cirugía endoscópica endonasal extendida a la región Petro-Clival / Ápex Petroso. Reporte de un caso y revisión de la literatura / Endonasal endoscopic surgery extended to the Petro-Clival / Petrópe region. Case report and literature review

INTRODUCCIÓN: las indicaciones de la cirugía endoscópica endonasal en el tratamiento de tumores de base de cráneo continúan expandiéndose, sobre todo para los tumores extradurales, como son los cordomas. A partir de un caso, nuestro objetivo fue revisar la literatura relevante de estos desafiantes tumores operados bajo esta técnica. CASO CLÍNICO: paciente de 59 de edad con antecedentes de depresión mayor, que comenzó con un cuadro de diplopía por compromiso del VI par izquierdo. Los estudios (CT y RM) mostraron un proceso expansivo en la región del ápex petroso. Se realizó un abordaje endoscópico extendido a la región petro-clival con resección tumoral subtotal. Buena evolución postquirúrgica con desaparición de su diplopía. El diagnóstico histológico fue de Cordoma Condroide. Se indicó radioterapia adyuvante. DISCUSIÓN: a partir del conocimiento y la experiencia en el manejo endoscópico de la patología intraselar se desarrollaron abordajes para el tratamiento de patologías que comprometen la fosa anterior, media e inclusive la fosa posterior. Actualmente, los abordajes endoscópicos, se han extendido a otras áreas de la base de cráneo que de otro modo presentan un reto técnico para la exposición a través de los abordajes transcraneales habituales. El abordaje endoscópico endonasal ofrece una ruta quirúrgica adecuada para la resección del tumor que se presenta en este caso. Las vías que pueden ser utilizados para llegar a la región petroclival a través de la acceso endonasal incluyen la medial (con o sin la movilización de ACI) y la infrapetrosa transterigoidea. En este reporte de caso se analizan las indicaciones del abordaje endoscópico endonasal basado en una revisión de la literatura. CONCLUSIÓN: el abordaje endoscópico endonasal extendido se presenta como una alternativa segura para el tratamiento de determinadas lesiones petro-clivales. Se requieren más estudios anatómicos y clínicos para establecer mejor el rol de este tipo de técnicas en el manejo de las lesiones localizadas en esta región

INTRODUCTION: indications for endoscopic endonasal surgery for the treatment of skull base tumors continue to expand, particularly for extradural tumors, such as chordomas. Based on this case report presentation, we aim to review the literature on the endoscopic technique relevant to the management of these challenging tumors. CASE REPORT: a 59 year-old woman who presented with diplopia due to left sixth nerve palsy underwent imaging studies (CT, MRI) that revealed a mainly intra-osseous expansive process of the left petrous apex. An expanded endoscopic endonasal approach to the petroclival region was performed and the tumor was subtotally resected. The patient recovered from surgery with resolved diplopia. Histopathology was compatible with chondroid chordoma. The radiation therapy was indicated after surgery. DISCUSSION: built upon the bulk experience on the treatment of intrasellar pathology, endonasal endoscopic approaches have been developed for the treatment of skull base lesions involving the anterior, middle and even posterior cranial fossae. Nowadays, the use of these techniques has spread to other areas of the skull base, which otherwise present as a formidable technical challenge for exposure through transcranial approaches. The endonasal endoscopic approach provides an adequate surgical corridor for the resection of the tumor presented in this case report. The alternative corridors that can be utilized to reach the petroclival region through the endonasal endoscopic route include the medial corridor (with or without ICA mobilization) and the transpterygoid infrapetrous corridor. In this article, we discuss the indications for the endoscopic endonasal approach for the case presented, and discuss our choice of approach based on our review of the literature. CONCLUSION: the extended endoscopic endonasal approach presents as a safe alternative for the treatment of select petroclival lesions. Further anatomical and clinical studies are required to better establish the role of the endoscopic endonasal approach for lesions located in this region

Cordoma de región sacra: comunicación de un caso / Chordoma in sacra region: report of a case

Estudio de caso: Paciente de sexo masculino de 77 años. Consultó por dolor lumbar. Una radiografía directa de columna vertebral reveló a nivel sacro, una voluminosa lesión solitaria osteolítica. El material fue procesado con la técnica de inclusión en parafina, coloración con H-E, P.A.S. y tricrómico de Masson.

Case Study: Male patient 77 years. He consulted by back pain. A direct X-column sacral spine revealed a voluminous solitary lesion osteolytic. The material was processed with the technique paraffin embedding, HE staining, PAS Y Masson trichrome.

Evolution of Minimally Invasive Approaches to the Sella and Parasellar Region

Introduction: Given advancements in endoscopic image quality, instrumentation, surgical navigation, skull base closure techniques, and anatomical understanding, the endonasal endoscopic approach has rapidly evolved into a widely utilized technique for removal of sellar and parasellar tumors. Although pituitary adenomas and Rathke cleft cysts constitute the majority of lesions removed via this route, craniopharyngiomas, clival chordomas, parasellar meningiomas, and other lesions are increasingly removed using this approach. Paralleling the evolution of the endonasal route to the parasellar region, the supraorbital eyebrow craniotomy has also been increasingly used as an alternative minimally invasive approach to reach this skull base region. Similar to the endonasal route, the supraorbital route has been greatly facilitated by advances in endoscopy, along with development of more refined, low-profile instrumentation and surgical navigation technology. Objectives: This review, encompassing both transcranial and transsphenoidal routes, will recount the high points and advances that have made minimally invasive approaches to the sellar region possible, the evolution of these approaches, and their relative indications and technical nuances. Data Synthesis: The literature is reviewed regarding the evolution of surgical approaches to the sellar region beginning with the earliest attempts and emphasizing technological advances, which have allowed the evolution of the modern technique. The surgical techniques for both endoscopic transsphenoidal and supraorbital approaches are described in detail. The relative indications for each approach are highlighted using case illustrations. Conclusions: Although tremendous advances have been made in transitioning toward minimally invasive transcranial and transsphenoidal approaches to the sella, furtherwork remains to be done. Together, the endonasal endoscopic and the supraorbital endoscope-assisted approaches are...

Nuances in the Treatment of Malignant Tumors of the Clival and Petroclival Region

Introduction: Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives: The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnostic identification, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to the skull base. Data Synthesis A literature review was conducted, searching for basic science and clinical evidence from PubMed, Medline, and the Cochrane Database. The selection criteria encompassed original articles including data from both basic science and clinical literature, case series, case reports, and review articles on the etiology, diagnosis, treatment, and management of skull base malignancies in the clival and petroclival region. Conclusions: The management of petroclival malignancies requires a multidisciplinary team to deliver the most complete surgical resection, with minimal morbidity, followed by appropriate adjuvant therapy. We advocate the combination of endoscopic and open approaches (traditional or minimally invasive) as required by the particular tumor followed by radiation therapy to optimize oncologic outcomes...

Acesso endoscópico endonasal para cordomas do clivus: relato de caso e revisão da literatura / Endoscopic endonasal approach for clival chordomas – case report and review of literature

Cordomas do clivo são lesões agressivas que se originam de remanescentes da notocorda primitiva, apresentando diagnostico e conduta desafiadoras. A ressecção radical da lesão tem sido recomendada para melhor prognóstico e o tratamento cirúrgico constitui desafio ao neurocirurgião. Vias de acesso transbasal, orbitozigomática, subtemporal, transcondilar, transmaxilar, e transesfenoidal extendida tem sido opções no tratamento cirúrgico. Relatamos caso de cordoma de clivus tratado por via endoscópica endonasal transesfenoidal.