Sacral Chordoma: Surgery Versus Definitive Radiation Therapy in Primary Localized Disease

Inclusion Criteria:

• Histologically confirmed diagnosis (brachyury expression) of primary sacralchordoma,of any diameter and arising at any site from S1 to coccyx.

• Age≥18years

• ECOG-performance status (PS) 0-2

• No previous antineoplastic therapy

• Macroscopic tumor detectable at MRI/CT scan

• Patient amenable for surgery

• Patient amenable for RT

• Written informed consent given before the enrolment, according to InternationalConference on Harmonisation/good clinical practice (ICH/GCP).

Exclusion Criteria:

• Distant metastasis

• Inability to maintain treatment position

• Prior radiotherapy to the pelvic region

• Prior therapy for sacral chordoma (including surgery, cryoablation, hyperthermia,etc)

• Local conditions that increase the risk of RT toxicity (tumor ulcerated skininfiltration, non-healing soft tissue infection, fistula in treatment field)

• Rectal wall infiltration

• General conditions that increase the risk of RT toxicity (active sclerodermia,xeroderma pigmentosum, cutaneous porphyria)

• Presence of a second active cancer (with the exception of non-melanoma skin cancerin-situ cervix neoplasia and other in-situ neoplasia)

• Severe comorbidities resulting in a prognosis of less than 6 months

• Inability to give informed consent

• Other malignancy within the last 5 years

• Performance status ≥ 2 (ECOG).

• Significant cardiovascular disease (for example, dyspnea > 2 NYHA)

• Significant systemic diseases grade >3 on the NCI-CTCAE v4.03 scale, that limitpatient availability, or according to investigator judgment may contributesignificantly to treatment toxicity

• Women who are pregnant or breast-feeding

• Psychological, familial, social or geographic circumstances that limit the patient'sability to comply with the protocol or informed consent