Rare and aggressive, chordomas are relatively slow-growing, invasive, and locally destructive tumors. They arise from cellular remnants of primitive notochord that extends rostrally from the sella turcica/spheno-occipital junction to the sacrum caudally. Their microscopic features include nests and cords of physaliphorous cells in a myxofibrillary stromal background.

The incidence of chordomas is 0.2 to 0.5 per 100,000 persons per year, and they account for 0.15% of all intracranial tumors.¹ They constitute 3 to 4% of all primary bone tumors. Within the sacrum, however, more than half of all primary bone tumors are chordomas.² Most commonly, they appear in patients over 30 years of age, and fewer than 5% of these tumors present in children.^2,​3 The male-to-female ratio is 1.8:1.

The three primary locations at which chordomas arise are sacrococcygeal (45–50%), spheno-occipital (35–39%), and in the vertebral column (15%). But this distribution has been disputed by McMaster and associates who, on the basis of their epidemiologic U.S.-based study, suggest that the actual distribution is roughly equal: 32% cranial, 32.8% spinal, and 29.2% sacral.⁴ They also suggest that the sacral location is more common in males and the cranial in females and African Americans.

Chordomas are prone to local aggressiveness, recurrence, and regrowth even after radical resection. Distant and cerebrospinal metastases, as well as surgical seeding, are common, and these tumors are relatively resistant to chemotherapy and radiotherapy ( ▶ Fig. 6.1, ▶ Fig. 6.2, ▶ Fig. 6.3, ▶ Fig. 6.4, ▶ Fig. 6.5, ▶ Fig. 6.6, ▶ Fig. 6.7, ▶ Fig. 6.8). Thus, radical resection should be the initial goal of treatment, if it is feasible. Surgical treatment for residual and recurrent disease is more difficult, however, because of the abundance of scar tissue and difficulty in establishing tissue planes. Regardless of the patient’s age and the location and invasiveness of the tumor, radical surgery is recommended.^5,​6 It is usually followed by high-dose proton beam radiotherapy, which aims to sterilize the postoperative field or treat the residual tumor.

Morbidity and mortality are more often associated with local tumor recurrence and progression than with metastasis. All of these characteristics make treatment and cure more difficult to achieve.

6.2 Local Aggressiveness and Progression

Despite radical surgical resection, local recurrence of chordomas ranges from 43 to 85%.^{5,​6,​7,​8} Large series of patients with sacral chordomas have shown that wide surgical margins are associated with increased survival and a decreased incidence of local recurrence regardless of the extent of local invasion.^{5,​6,​7,​8} In their series, Ruggieri and colleagues reported that 55% of their patients had wide-margin resections, 16% had marginal resections, 5% had intralesional, and 19% had wide contaminated margins.⁷ The overall survival rate was 97% at 5 years, 71% at 10 years, and 47% at 15 years, with an overall metastasis rate of 30%. Patients with less than wide surgical margins had increased local recurrence rates. Surgical resection with wide margins has been associated with a local recurrence rate of 5 to 17% compared with 71 to 81% when margins were intralesional or marginal.⁷ In their study of 62 patients over 11 years treated with proton beam irradiation, O’Connell and colleagues reported that female sex, tumor necrosis seen on a preradiation biopsy, and a tumor volume greater than 70 cc were independent predictors of a shortened overall survival.⁹ Kilgore and Prayson, in their study of apoptotic and proliferative markers in chordomas, concluded that low MIB-1 labeling index and the lack of p53 and Bcl-2 staining are consistent with the low-grade nature of the tumor.¹⁰

In their U.S.-based epidemiologic study, McMaster and associates reported a median survival of 6.29 years, and 5- and 10-year survival rates of 68% and 40%, respectively.⁴ They observed no difference in survival with regard to the patients’ sex or the tumor’s site of presentation. Bergh and colleagues, however, report that patients with lumbosacral tumors have a longer mean survival (8–10 years) as opposed to those with a tumor at the skull base (4–5 years).¹¹ From their experience, Menezes and coworkers concluded that gross total resection, incomplete resection, and repeated resection or proton beam irradiation for recurrence are treatment options.¹

In their series of 23 skull base chordomas (10 radical, 11 subtotal, and 2 partial resections), Al-Mefty and Borba noted that radical resection followed by high-dose proton radiation led to a mean disease-free interval of 14.4 months.¹² The choice of radical resection followed by radiation was also supported by Heffelfinger and associates.¹³

Gay and associates have reported a 5-year recurrence-free survival rate of 65% in their skull base series.¹⁴ Patients who underwent previous surgery had a greater risk of tumor recurrence than patients who did not have surgery—a 5-year recurrence-free survival rate of 64% compared with 93% for those having no prior surgery. They noted that the survival rate for patients who had total or near-total resection was significantly higher than that of patients with subtotal or partial resections. Their conclusion is that total or near-total resection should be the preferred treatment.¹⁴

Raffel and colleagues, in their study of cranial chordomas in 26 patients, suggested that the median survival was 4.2 years.¹⁵ They also suggested that radical tumor resection and postoperative high-dose radiation contributed to better outcomes.

In a study of 51 patients with cranial chordomas operated on at the Mayo Clinic over a 24-year period, Forsyth and associates reported that patients who underwent resection lived longer, with a 36% versus 55% 5-year survival rate for biopsy versus resection.¹⁶ In addition, those who received postoperative radiation therapy had longer disease-free survival, and those with the chondroid subtype had the same survival rates as those with typical chordomas. Furthermore, a younger patient age, the presence of diplopia, and the absence of mitotic activity were associated with longer survival.¹⁶

Borba and colleagues reviewed their results and the literature regarding the outcomes of chordoma treatment in children. The lesions in children younger than 5 years had a higher prevalence of atypical histologic chordoma patterns, greater cellularity, more aggressive behavior, no chondroid component, a higher rate of metastasis, and overall worse prognosis¹⁷ ( ▶ Fig. 6.4). The prognosis of children older than 5 years with chordomas was no different than in the adult population. Patients treated through radical tumor resection followed by radiation fared significantly better than those treated with radiation alone.¹⁷ Wold and Laws also reported their experience at Mayo with 12 patients under the age of 19 who had clival chordomas. After partial or radical resection and subsequent radiation, patients with chondroid chordomas had a worse prognosis than those with typical chordomas, a finding that differs from the adult population.¹⁸

In another series from the Mayo Clinic of sacral chordomas, the overall survival rates were 74, 52, and 47% at 5, 10, and 15 years, respectively, and the most important predictor of survival and recurrence was a wide margin of resection. Forty-four percent of patients had local recurrence, and the recurrence-free survival was 59% and 46%, respectively, at 5 and 10 years after an average follow-up of 7.8 years.¹⁹

Related posts:

Surgical Decision Making in Chordomas Molecular Imaging of Chordomas Radiation Treatment for Chordomas Frontobasal Approaches to Clival Chordomas The Extended Petrosal Middle Fossa Approach Chordomas: A Personal Perspective

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