Natural history and surgical treatment of chordoma: a retrospective cohort study

Sao Paulo Med J. 2014;132(5):297-302. doi: 10.1590/1516-3180.2014.1325628. Epub 2014 Jul 29.

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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Brazil / epidemiology
  • Child
  • Child, Preschool
  • Chordoma / mortality*
  • Chordoma / radiotherapy
  • Chordoma / surgery
  • Female
  • Humans
  • Male
  • Medical Records
  • Middle Aged
  • Neoplasm Recurrence, Local / mortality
  • Retrospective Studies
  • Sacrum*
  • Skull Base Neoplasms / mortality*
  • Skull Base Neoplasms / radiotherapy
  • Skull Base Neoplasms / surgery
  • Spinal Neoplasms / mortality*
  • Spinal Neoplasms / radiotherapy
  • Spinal Neoplasms / surgery
  • Survival Rate
  • Treatment Outcome
  • Young Adult