ABSTRACT
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. .
Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Chordoma/mortality , Sacrum , Skull Base Neoplasms/mortality , Spinal Neoplasms/mortality , Brazil/epidemiology , Chordoma/radiotherapy , Chordoma/surgery , Medical Records , Neoplasm Recurrence, Local/mortality , Retrospective Studies , Skull Base Neoplasms/radiotherapy , Skull Base Neoplasms/surgery , Spinal Neoplasms/radiotherapy , Spinal Neoplasms/surgery , Survival Rate , Treatment OutcomeSubject(s)
Humans , Adult , Central Nervous System Neoplasms , Chordoma , Glomus Jugulare , Neuroma, Acoustic , Central Nervous System Neoplasms/diagnosis , Central Nervous System Neoplasms/rehabilitation , Chordoma/surgery , Chordoma/diagnosis , Chordoma/radiotherapy , Meningioma/surgery , Meningioma/diagnosis , Meningioma/epidemiology , Neuroma, Acoustic/diagnosis , Skull BaseABSTRACT
Chordomas are difficult and challenger tumors to treat. Despite of its pathological appearance that demonstrates an slow-growing behavior, their location and patterns of spread through the base of the skull make these tumors one of the most difficult diseases to treat at the base of the skull. The inadequate response to the conventional radiation therapy and the high incidence of recurrence, in cases treated with classical neurosurgical approaches, make mandatory a radical removal of the tumor mass and the surrounding pathological bone which can only be achieved with the use of skull base approaches associated with an extensive drilling of the bony tumor. In order to reach the goal of the surgical treatment of the skull base chordomas, the mastery of the skull base anatomy and the management of the drill are crucial points to obtain a successful results. In this report, we express our philosophy to treat these tumors which is based on the radical removal associated to a postoperative proton beam therapy. The main characteristic of the skull base chordomas is to spread through the base of the skull, which dictates the surgical approach that should be employed for each patient. In this article we will address the management of the skull base chordomas, with special emphasis to the role of the surgical and radiation treatment. The pathologic diagnosis and the radiological findings of chordomas of the skull base also will be discussed.
Subject(s)
Humans , Chordoma/surgery , Skull Base Neoplasms/surgery , Chordoma/diagnosis , Chordoma/radiotherapy , Skull Base Neoplasms/diagnosis , Skull Base Neoplasms/radiotherapyABSTRACT
los cordomas son neoplsias que se originan de remanentes de la notocorda. Su frecuencia es mayor en adultos y se ha informado de 13 casos en edades pediátricas hasta 1988, en la literatura internacional y ninguna en la nacional. Se presentan 3 casos tratados en nuestro hospital de 1984 a 1988, los tres casos corresponden a mujeres con edades de 11,7 y 16 años; el primero presentó el tumor localizado ventralmente al clivus con invasión a órbita y el segundo y tercer caso con localización dorsal al mismo