Chordoma: retrospective analysis of 24 cases

CASE REPORT

Chordoma: retrospective analysis of 24 cases

Ademar Lopes; Benedito Mauro Rossi; Claudio Regis Sampaio Silveira; Antonio Correa Alves

A. C. Camargo Hospital, Antonio Prudente Founfation - São Paulo, Brazil

^{Address for correspondence}

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.

Uniterms: Chordoma, sacral tumor, notochord, bone tumor, treatment.

RESUMO

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.

INTRODUCTION

Chordoma was first described by Virchow in 1857 under the name of "ecchordosis physaliphora".¹⁷ In 1894, Ribbert defined chordoma as a malignant tumor arising from embryonic remains of the notochord.¹²

The preferential localization of chordoma is in the vertebral column, from the spheno-occipital synchondrosis to the coccyx, more frequently in the sacrococcygeal region.⁹

The majority of chordomas are slow growing with local proliferation and high aggressiveness and infiltration of the adjacent organs and structures (Fig. 1). Surgery is the only curative treatment^7,8,9,13 when adequate margins are obtained. There is a low rate of metastases for this type of tumor.⁷

MATERIALS AND METHODS

A series of 24 patients with chordomas was identified in the Hospital files (1953-1993), with ages ranging from 2 to 86 years (mean=34.5), 12 males and 12 females; 22 were white. The anatomical location was: sacrum-20 cases (83.4%), 8 with extension to the coccyx and 5 invading to the lumbar column; head and neck-3 cases (12.4%) in the clivus region; retroperitoneum- 1 case (4.2%). A summary of clinical data, treatment and follow-up is shown in Table I.

When ultrasonography, computerized tomography and magnetic resonance were not available, the diagnosis of tumors in the sacral region were made through digital rectal examination and barium enema. In the absence of imaging exams, the diagnosis of chordomas of other locations were very difficult due to the lack of other trustworthy methods.

Our therapeutic approach for chordomas is curative surgery followed by adjuvant radiation therapy and/or chemotherapy. This conduct was not always possible in our series because 13 out 24 cases were previously submitted to various types of treatment with residual or recurrent disease, and the majority of the intact cases had advanced tumors. In two cases, due to this reason, no treatment was possible.

Radiation therapy, combined or alone, was the treatment in 16 (66.7%) of the cases, being 9 (37.5%) associated with surgery and 5 (20.9%) with chemotherapy. Chemotherapy was the exclusive treatment in the case of the retroperitoneal tumor.

RESULTS

In the 20 cases with tumor in the sacral region, the treatment and overall survival were: a) radiation therapy alone (5 cases) - 5 to 50 months (mean-18.8); b) radiation therapy and surgery (4 cases) - 1 to 88 months (mean-27.8); c) radiation therapy, chemotherapy and surgery (4 cases) - 6 to 45 months (mean-24.8); d) surgery alone (3 cases) - 11 to 17 months (mean-14); e) chemotherapy and surgery (1 case) - 27 months; f) chemotherapy and radiation therapy (1 case) - 31 months. Two patients did not receive any treatment due to the advanced stage of the disease.

In the 3 cases with tumors in the head and neck region (ages 10,11 and 21 years old), the first was treated with surgery and radiation therapy and has been disease free for a 21-month period; the second was submitted exclusively to radiation therapy, dying due to the disease after 11 months, and the third was treated with surgery alone and has been disease free for a 3-month period.

The patient with tumor in the retroperitoneum was submitted exclusively to chemotherapy, dying 11 months after admission.

From the total of 24 cases, eight (33.4%) have developed metastases in the following sites: lung- 5; liver, lymphonodus and bone- one case each. The 5-year overall survival is shown in Figure 2.

Chordoma is a rare neoplasm even in a cancer center. The present series of 24 cases represented 0.02% of all 79,917 new cancer cases seen at the A.C.Camargo Hospital (1953-1993). Among the 1,030 cases of bone tumors in the same period, chordomas corresponded only to 2.2%.

The chordoma can arise from all axial skeleton, hut various authors show that 50% begin in the sacrum, 35% in the spheno-occipital region and 15% in the vertebrae.^3,6,7,19 The localization out of the spine is rare.⁹ In this series, the majority of the tumors were in the sacral region, but we must remember that before the introduction of computerized tomography, it was difficult to obtain the diagnosis of spheno-occipital lesions.

The time elapsed between the initial symptoms and the treatment is usually very long, which is a characteristic of a slow-growing tumor. Presence of mass and sacral pain, with rectal and urinary bladder dysfunction are the main symptoms of the disease in the sacrococcygeal region.¹⁵ The digital rectal examination is a very important propedeutic method for diagnosis of sacral tumors.

In spheno-occipital lesions the main signs and symptoms are pain, mass-invading nasopharynx, sometimes with obstruction of air passage and dysfunction of cranial nerves.⁸

The metastases rate found in our series (33.4%) is within the range described in the literature.^4,8,9,18

Conventional x-ray, computerized tomography and magnetic resonance are very important in the diagnosis and to plan the better treatment. In the histopathology, the presence of large cells with lobular arrangement and intracytoplasmatic mucus, called physaliphora cells, is characteristic of chordomas (Fig. 3).

Surgery with adequate margins is the best available treatment for chordomas, but this is not always possible due to the advanced stage of the disease and difficult access⁵ (Fig. 4).

The extent of curative surgery is a dilemma, because the total removal of the tumor many times implies rectal and urinary bladder impairment.

The results of radiation therapy alone are not satisfactory, however is indicated in the absence of better therapeutic options^8,18and the dosage varies from 70 to 80 Gy.^11,16

The chemotherapy (PVB scheme - cisplatin, vinblastine, bleomycin) was used in our retroperitoneal case of chordoma and was unsuccessful. Azzarelli reported one case with complete remission with chemotherapy (PVB scheme) that allowed surgical treatment. However, this patient had local recurrence 3 months later.²

A 50% five-year survival rate is reported by Smith in patients with sacral lesions.¹⁴ In the Queen Elizabeth Hospital series no difference was found between patients submitted to palliative radiation therapy and those without treatment.⁴ The same author reported a 5-year survival rate between 50% and 60% for cases submitted to surgery with adequate margins followed by radiation therapy, 25% for cases submitted to palliative radiation therapy and 15% for patients submitted to palliative surgery.⁴ Keisch reported a 5-year survival rate of 100% for cases submitted to surgery with adequate margins and radiation therapy, and 68% for cases submitted only to surgery with adequate margins.¹⁰ This excellent result indicates the necessity of combined treatment and early diagnosis. The five year overall survival reported by some authors are shown in Table 2.

The 4.2% five year survival obtained in our series is an indication of the advanced stage of the disease and recurrence after previous inadequate treatment.

Since chordomas are a slow-growing and rare tumors, multidisciplinary approach and early diagnosis are required for adequate treatment.

References

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• 2. Azzarelli A, Quagliuolo V, Cerasolli S et al Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 1985;37:185-91.
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• 8. Heffelfinger MJ, Dahlin DC, MacCarthy CS et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410-20.
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• 10. Keisch ME, Garcia DM, Shibuya RB. Retrospective long-term follow-up analysis in 21 patients with chordoma of various sits treated at a single institution. J Neurograf 1991;75:374-7.
• 11. Pearlman AW, Sigh RK, Hoppenstein R et al Chordoma: combined therapy with radiation and surgery: case report and new operative approach. Bull Hosp Joint Dis 1972;33:47-57.
• 12. Ribbert H. Ueber die Ecchordosis physalifora spheno-occipitalis. Zentralbl Allg Pathol 1894;5:457-61.
• 13. Rich TA, Schiller A, Suit HD, Mankin HJ. Clinica and pathologic review of 48 cases of chordoma. Cancer 1985;56:182-7.
• 14. Smith J, Ludwig RL, Marcove RC Sacrococcygeal Chordoma. Skeletal Radiol 1987; 16: 37-44.
• 15. Sundaresan N, Galich JH, Chu FCH et al. Spinal chordomas. J Neurosurg 1979;50:312-9.
• 16. Tewfik HH, McGinnis WL, Nordstrom DG et al. Chordoma: evaluation of clinical behavior and treatment modalities. Int J Radiat Oncol Biol Phys 1977;2:959-62.
• 17. Virchow RLK. Untersuchungen über die Entwickenlung des Schädelgrundes in gesunden und krakhaften Zustande und über den Einflus derselben auf Schädelform, Gesichtsbildung und gehirnbau - Berlim, G. Reimer, 1957. p.128.
• 18. Volpe R, Mazabraud A A clinicopathologic review of 25 cases of chordoma. Am J Surg Pathol 1983;7:161-70.
• 20. Welinger CL. Rachidial Chordoma: III Review of the literature since 1960. Rev Rhum Mal Osteoartic 1975;42:287-95.

Address for correspondence:

Ademar Lopes

Departamento de Cirurgia Pélvica

Hospital A.C.Camargo - Fundação Antonio Prudente

Rua Prof. Antonio Prudente, 211

São Paulo/SP - Brasil - CEP 01509-010

Publication Dates

• Publication in this collection
  06 Nov 2008
• Date of issue
  Dec 1996