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.

Rara presentación de polineuropatía distal hipoglicémica causada por un insulinoma en el contexto de una neoplasia endocrinamúltiple tipo 1 / An unusual presentation of hypoglycemic distal polyneuropathy caused by an insulinoma in the context of multiple endocrine neoplasia 1

La neoplasia endocrina múltiple tipo 1 es una enfermedad de rara presentación. Se caracteriza por el compromiso tumoral neuroendocrino, de paratiroides, hipófisis y enteropancreático. Presentamos el caso de una paciente de 19 años con síntomas de cefalea, convulsiones y debilidad de las cuatro extremidades. Se confirmó la presencia de hipoglicemia 33mg/dL. El estudio elecromiográfico evidenció polineuropatía sensitivo motora en las cuatro extremidades. La resonancia magnética abdominal mostró un tumor en la cola del páncreas que luego de la pancreatectomía se confirmó como insulinoma. La glicemia se normalizó. Además, presentó un macroadenoma hipofisario, hiperparatiroidismo primario y tumor adrenal no funcionante. A los 25 años presentó cefalea intensa y amaurosis de ojo derecho, en la tomografía axial se evidenció tumoración hipofisaria gigante y en estudio de patología se diagnosticó neoplasia maligna condroide (cordoma).

Multiple endocrine neoplasia type 1 is a rare disease. It is characterized by the neuroendocrine, parathyroid, pituitary, and enteropancreatic tumor involvement. We present the case of a 19 year old patient with symptoms of headache, convulsions and weakness of the four extremities. The presence of hypoglycemia 33mg/dL was confirmed. The electromyographic study showed motor sensory polyneuropathy in all four extremities. The abdominal magnetic resonance showed a tumor in the tail of the pancreas that after the pancreatectomy was confirmed as insulinoma. The glycemia was normalized. In addition, he presented a pituitary macroadenoma, primary hyperparathyroidism and non-functioning adrenal tumor. At 25 years of age, he presented severe headache and amaurosis of the right eye. Axial tomography showed a giant pituitary tumor and in the study of pathology chondroid malignancy (chordoma) was diagnosed.

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)

Consideraciones histopatológicas del tumor óseo cordoma

El Cordoma es una neoplasia maligna del tejido óseo, cuya localización es en la columna vertebral o en la base del cráneo de los seres humanos. Sus células recuerdan a las de la notocorda. Se postula que tiene su origen en restos notocordales, sin embargo la edad más frecuente de aparición es después de los 40 años en el sexo masculino. Los sitios de predilección son el sacro y la base del cráneo. En los periodos finales de su aparición existen trastornos de la micción, defecación y movilidad de los miembros inferiores, y trastornos visuales cuando su localización es en la base del cráneo. El tratamiento quirúrgico radical no es muy alentador por su localización anatómica, de ahí lo usual que resultan las recidivas. Las metástasis son poco frecuentes y casi siempre los órganos diana son los pulmones, menos frecuente en piel y ovario

The Chordoma is a malignant neoplasm of bone tissue, whose location is in the spine or at the base of the skull of human beings. His cells remind of the notochord. It is postulated that it has its origin in Notocordales remains; however the most frequent age of appearance is after 40 years in the male sex. The sites of predilection are the sacrum and the base of the skull. In the final periods of their appearance there are disorders of urination, defecation and mobility of the lower limbs, and visual disorders when their location is at the base of the skull. Radical surgical treatment is not very encouraging because of its anatomical location, hence the usual recurrences. Metastases are rare and almost always the target organs are the lungs, less common in skin and ovary

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 sacrococcígeo tratado com ressecção e radioterapia complementar: relato de caso / Sacrococcygeal chordoma treated with resection and radiotherapy: a case report

Cordoma sacrococcígeo que é uma neoplasia malignade grau baixo a intermediário, remanescente da notocorda.Apresenta crescimento indolente, entretanto comcomportamento local agressivo. Trata-se de uma neoplasiarara e que apresenta diagnóstico tardio visto suapobre sintomatologia no início da doença. A localizaçãoé mais comum no esqueleto axial, e neste relato descrevemoso tratamento de um cordoma sacrococcígeo.

Sacrococcygeal chordoma is a malign neoplasia oflow degree remainder of the notocorda. Presents indolentgrowth, meantime with aggressive local behavior. Itis a rare neoplasia and that it presents seen late diagnosishis poor symptomatology in the beginning of thedisease. The location is more common in the axial skeleton,and in this report we described the treatment of asacrococcygeal chordoma.

Cordoma sacral / Sacral chordomas

Cordomas são neoplasias raras que se originam dos remanescentes da notocorda primitiva 50% dos casos têm localização sacral, sendo mais frequente ao nível de S4/S5. Nós descrevemos um caso de cordoma sacral ao nível de S1 e discutimos a apresentação clínica, achados de imagem, tratamento cirúrgico e evolução.

Chordomas are rare neoplasias originating from the remaining primitive notochord. 50% of the cases have sacral localization, being more frequent at levels S4/S5. Here we report a case of sacral chordoma at level S1 and discuss its clinical presentation, imaging findings, surgical treatment, and progress.

Cordoma Sacrococcígeo gigante: relato de caso / Giant Sacrococcygeal chordoma: case report

Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.

Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive behavior, well-known resistance to radiation therapy, meaningful surgical morbimortality and increased recurrence rate become its treatment a challenge. We describe a case of a giant unresectable sacrococcygeal chordoma.

Cordomas gigantes del clivus: ¿Se justifican las cirugías extensas? / Giant clivus chordomas: Are extensive surgeries really justified?

Debido a su localizacion y comportamiento biológico, los cordomas del clivus son tumores que aún representan un gran reto, principalmente cuando han alcanzado grandes dimensiones. En este artículo, hemos seleccionado a los pacientes con los cordomas del clivus más grandes, a fin de analizar si realmente se pueden beneficiar con procedimientos quirúrgicos extensos, con la morbilidad que ello implica. Once pacientes con cordomas gigantes del clivus fueron incluidos, todos ellos intervenidos de acuerdo a los criterios recientes de cirugía de base de cráneo. Fue analizado principalmente si el grado de resección presentaba alguna relación con el estado funcional postoperatorio y el tiempo de supervivencia libre de enfermedad; también fue evaluado si existía alguna correlación entre los hallazgos histopatológicos y el curso clínico. En este grupo fue posible lograr una resección total del tumor en 8 pacientes y parcial en 3. En todos los casos se observó una evidente mejoría en el estado funcional postoperatorio, pero particularmente en los pacientes con resecciones totales. El tiempo de supervivencia libre de enfermedad fue también mayor en los pacientes con las resecciones más amplias. No se encontró correlación alguna entre la variedad histológica de los tumores (clásico o condroide) y el pronóstico clínico.

Because of their location and biological behavior, clivus chordomas are tumors that still represent a great challenge, mainly when they have reached huge dimensions. In this paper we have selected the patients with the biggest clivus chordomas to analyze if they really can be helped with the current surgical procedures with the morbidity that it implies. Eleven patients with giant clivus chordomas were included. All of them were operated on following the recent criteria of Cranial Base Surgery. It was mainly analyzed, if the degree of the resection had any relation to the postoperative functional outcome and the disease-free survival time; it was also evaluated if there existed a correlation between the pathologic findings and the clinical course. It was possible to get a total resection in 8 patients and partial in 3. In all cases there was an evident improvement in their postoperative functional outcome, specially in those with complete resections. The disease-free survival time was also greater in the patients with more ample resections. There was not any correlation among the histologic variety of tumors (typical or chondroid) andthe clinical prognosis.