Chordoma: retrospective analysis of 24 cases

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Abstract

INTRODUCTION: Chordoma is a rare and slow-growing tumor, with local aggressiveness and preferential localization in the vertebral column. OBJECTIVE: The main objective of this study is to evaluate natural history and results of treatment of chordomas. METHODOLOGY: This is a retrospective study from 1953 to 1993. MATERIAL AND METHODS: The age ranged from 2 to 86 years (mean=34.5). Twelve patients were male and 12 female. The localization of the tumor was: 20 in the sacral region, 3 in head and neck and one out of the spine. RESULTS: The treatment, alone or combined, was surgery, radiation therapy and chemotherapy. The survival rate for patients with lesions in the sacrum ranged from 4 to 119 months, since the date of the symptoms. The 5-year overall survival was 4.2%. CONCLUSION: Chordoma is a rare and slow growing tumor, with a very difficult approach by surgery due to its preferential location in the sacrum and poor therapeutic results with radiation therapy or chemotherapy, mainly in patients with advanced disease.

Translated abstract

INTRODUÇÃO: O cordoma é um tumor raro e de crescimento lento, com agressividade local e localização preferencial na coluna vertebral. OBJETIVO: O objetivo principal deste estudo e analisar a historia natural e os resultados de tratamento dos cordomas. METODOLOGIA: Este é um estudo retrospectivo realizado entre 1953 e 1993. MATERIAL E MÉTODOS: A idade variou de 2 a 86 anos (média=34,5). Doze pacientes eram do sexo masculino e 12 do sexo feminino. A localização do tumor era: região sacral em 20 casos, cabeça e pescoço em 3 casos e fora da coluna em um caso. RESULTADOS: O tratamento, sozinho ou combinado, foi cirurgia, radioterapia e quimioterapia. A sobrevida para pacientes com lesões no sacro variou de 4 a 119 meses, desde a data dos sintomas. A sobrevida global em 5 anos foi de 4,2%. CONCLUSÃO: Cordoma é um tumor raro e de crescimento lento, com uma abordagem terapêutica cirúrgica difícil devido a sua localização preferencial no sacro e resultados pobres com radioterapia ou quimioterapia, principalmente em pacientes com doença avançada.

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Most cited references 19

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Chordomas and cartilaginous tumors at the skull base.

M Heffelfinger, D C DAHLIN, C MacCarty … (1973)

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Clinical and pathologic review of 48 cases of chordoma.

H D Suit, H Mankin, Matthew Rich … (1985)

The results of treatment of 48 patients with the diagnosis of chordoma during the period 1931 to 1981 at the Massachusetts General Hospital were reviewed. Fourteen patients were treated with surgery alone: eight patients with primary tumors in the sacrococcygeal region were treated with radical surgery and four are alive with no evidence of disease (NED) with follow-up of 8 to 20 years. Recurrent tumors in six patients were treated with surgery alone resulting in long palliation (3-25 years). The actuarial survival rate at 5 years for all patients treated with surgery was 76%. Radiation therapy was used in patients after either a biopsy (15), partial excision (17), or before radical excision in 2 patients. To achieve a worthwhile level of palliation, doses greater than 4000 cGy were required. High-dose levels (greater than 6500 cGy) were achieved in nine cases by a combination of photon and 160 MeV proton beams. The results to date of this approach for lesions of the base of skull and cervical vertebral body are encouraging: high local control and low morbidity. The 5-year actuarial survival rate of all patients treated with radiation was 50%.