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Case report The authors report a case of an 11-year-old boy that presented with headache and vomiting that was present for several months. CT and MR imaging revealed a large prepontine mass and an obstructive hydrocephalus. A ventriculoperitoneal shunt was inserted, and in a second operation, a radiologically proven total resection was performed, using a left frontotemporal transsylvian approach. The tumour showed no involvement of the dura or clivus. Histological examination showed the characteristics of a chordoma. No further adjuvant treatment was given. The patient remained disease or tumour free after a 6-year follow-up. Discussion Intradural chordomas are extremely rare tumours that originate from notochordal remnants. Only three other cases have been reported in the paediatric population. Ecchordosis physaliphora (EP) is an ectopic notochordal remnant that has a similar biological behaviour and is difficult to distinguish from intradural chordomas. They might exist in a continuum from benign notochordal tumour to malignant chordoma. A surgical resection without adjuvant radiation therapy is suggested to be the treatment of choice in the paediatric population. Conclusion The authors describe a rare case of an intradural prepontine chordoma in an 11-year-old boy that stayed disease free after a 6-year follow-up.

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Most cited references 29

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Chordoma: incidence and survival patterns in the United States, 1973-1995.

M L McMaster, A Goldstein, C Bromley … (2001)

Chordoma, a rare tumor arising from notochordal remnants, has been described to date only by single-institution case series or small population-based surveys. We used data from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute, 1973-1995, to calculate age-adjusted incidence and survival rates for 400 cases of microscopically confirmed chordoma and to derive information regarding case distribution and risk of second cancer. The age-adjusted chordoma incidence rate (IR) of 0.08 per 100,000 was age-dependent, more common in males (IR 0.10) than females (IR 0.06) and rare among patients aged <40 years and blacks. Within the axial skeleton 32% of cases were cranial, 32.8% spinal and 29.2% sacral. Young age (<26 years; p = 0.0001) and female sex (p = 0.037) were associated with greater likelihood of cranial presentation. There was no overall increased risk for second primary cancers after chordoma. Median survival was 6.29 years; 5- and 10-year relative survival rates were 67.6% and 39.9%, respectively. Comparison with other bone sarcomas revealed racial disparities in incidence for the two developmental tumors, chordoma and Ewing's sarcoma. This study provides new data regarding incidence and survival patterns of chordoma in the US. Additional epidemiologic studies are required to elucidate the genetic and environmental determinants underlying this rare, distinctive neoplasm.

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Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients.

L.-G. Kindblom, F Remotti, B Gunterberg … (2000)

The prognosis of patients with chordoma of the sacrum and mobile spine has been reported to be dismal and attributable in the majority of cases to intralesional surgery. The purpose of this study was to evaluate the clinical outcome of these patients using modern surgical principles aimed at complete resection and to identify prognostic factors. The clinical and morphologic features, type of surgery, and follow-up of 39 consecutive patients with chordoma were reviewed and analyzed statistically. Thirty sacral and 9 mobile spine chordomas (size range, 3-20 cm; mean, 8 cm) occurring in 22 women and 17 men (median age, 55 years) were analyzed. The preoperative morphologic diagnosis was based on fine-needle aspiration (FNA) biopsy, core needle biopsy, or incisional biopsy. The final surgical margins were wide in 23 patients and marginal or intralesional in 16. The mean follow-up was 8.1 years (range, 0.1-23 years). Seventeen patients (44%) developed local recurrences and 11 patients (28%) developed metastases. The estimated 5-, 10-, 15-, and 20-year survival rates were 84%, 64%, 52%, and 52%, respectively. Local recurrence was associated significantly with an increased risk of metastasis and tumor-related death (P 5%, and local recurrence were found to be adverse prognostic factors. FNA is the preferred method for establishing the preoperative morphologic diagnosis of chordoma.

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Intracranial chordomas: a clinicopathological and prognostic study of 51 cases.

D Piepgras, P A Forsyth, C. E. Shaw … (1993)

Fifty-one patients with intracranial chordomas who were surgically treated between 1960 and 1984 were studied. Median patient age was 46 years, and 73% presented with diplopia or headache. Nineteen tumors were classified as the "chondroid" type. The extent of surgical removal was a biopsy in 11 patients and subtotal removal or greater in 40. Thirty-nine patients received postoperative radiation therapy. At the time of analysis, 17 patients were alive, and the estimated 5- and 10-year survival rates were 51% and 35%, respectively, for the group of 51 patients. Univariate analysis showed that: 1) patients undergoing resection lived longer (the 5-year survival rate was 36% for the 11 biopsy patients compared with 55% for the 40 patients who had resection; 2) patients who underwent postoperative radiotherapy tended to have longer disease-free survival times; and 3) overall and disease-free survival data were the same for patients with chondroid tumors and those with typical chordomas. Multivariate analysis showed that: 1) age was the factor most strongly associated with longer overall and disease-free survival; 2) diplopia was associated with longer survival; and 3) tumoral mitotic activity tended to be associated with shorter disease-free survival. One tumor metastasized to the cervical cord, and two tumors underwent anaplastic transformation. These data suggest that the prognosis in patients with chordomas is unfavorable, young age is the single factor most strongly associated with longer survival, surgical resection is beneficial, and postoperative radiotherapy may prolong disease-free survival.

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PubMed ID:: 26216058

PMC ID:: 4735251

DOI:: 10.1007/s00381-015-2818-z

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ScienceOpen disciplines: Neurology

Keywords: chordoma,intradural,prepontine,paediatric

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ScienceOpen disciplines: Neurology

Keywords: chordoma, intradural, prepontine, paediatric

Intradural prepontine chordoma in an 11-year-old boy. A case report

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Karger: Neurology and Neuroscience

Most cited references 29

Chordoma: incidence and survival patterns in the United States, 1973-1995.

Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients.

Intracranial chordomas: a clinicopathological and prognostic study of 51 cases.

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