Chordoma
retrospective analysis of 24 cases
Keywords:
Chordoma, sacral tumor, notochord, bone tumor, treatmentAbstract
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.
Downloads
References
Amendola MA, Pover E, McLachey KD Chordoma: role of radiation therapy. Radiology 1986;158:839-43.
Azzarelli A, Quagliuolo V, Cerasolli S et al Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 1985;37:185-91.
Bethke KP, Neifeld JP, Lawrence Jr.W Diagnosis and management ofsacrococcygeal chordoma. J Surg Oncol 1991; 48: 232-8.
Chetiyawardana AD. Chordoma: results of treatment. Clin Radiol 1984;35:159-61.
Chetty R, Levin CV, Kalan MR Chordoma: a 20-year clinicopathologic review of the experience at Groote Schuur Hospital, Cape Town. J Surg Oncol 1991;46:261-4.
Dahlin DC, MacCarty CS. Chordoma: a study of fifty-nine cases. Cancer, 1952;5:1170-8.
Gentil FC, Coley BL Sacrococcygeal chordoma. Ann Surg 1948;127:432-55.
Heffelfinger MJ, Dahlin DC, MacCarthy CS et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410-20.
Higinbotham NL, Phillips RF, Farr HW et al. Chordoma: thirty-five-year study at Memorial Hospital. Cancer 1967;20:1841-50.
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.
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.
Ribbert H. Ueber die Ecchordosis physalifora spheno-occipitalis. Zentralbl Allg Pathol 1894;5:457-61.
Rich TA, Schiller A, Suit HD, Mankin HJ. Clinica and pathologic review of 48 cases of chordoma. Cancer 1985;56:182-7.
Smith J, Ludwig RL, Marcove RC Sacrococcygeal Chordoma. Skeletal Radiol 1987; 16: 37-44.
Sundaresan N, Galich JH, Chu FCH et al. Spinal chordomas. J Neurosurg 1979;50:312-9.
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.
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.
Volpe R, Mazabraud A A clinicopathologic review of 25 cases of chordoma. Am J Surg Pathol 1983;7:161-70.
Welinger CL. Rachidial Chordoma: III Review of the literature since 1960. Rev Rhum Mal Osteoartic 1975;42:287-95.