Chordoma Guidelines

Updated: Nov 10, 2016
  • Author: Cheryl Ann Palmer, MD; Chief Editor: Brian H Kopell, MD  more...
  • Print
Guidelines

Guidelines Summary

Guidelines Contributor: Mrinal M Gounder, MD Attending Physician in Medical Oncology, Sarcoma and Developmental Therapeutics Service, Memorial Sloan-Kettering Cancer Center

Guidelines for the management of chordoma have been published by the following organizations:

  • National Comprehensive Cancer Network (NCCN) [27]
  • European Society for Medical Oncology (ESMO) [28]

Diagnosis

National Comprehensive Cancer Network (NCCN) guidelines recommend that all patients younger than 40 years with abnormal radiographs be referred to an orthopedic oncologist for further workup that includes biopsy. For patients 40 years of age or older, the recommended workup includes the following [27] :

  • Computed tomography (CT) of the chest, abdomen, and pelvis
  • Bone scan
  • Mammogram and other imaging studies as clinically indicated

Findings of other lesions indicates a non-bone primary tumor. If no other lesions are found, the patient should be referred to an orthopedic oncologist for a biopsy. [27]

European Society for Medical Oncology (ESMO) guidelines recommend follow-up of an abnormal radiograph with magnetic resonance imaging (MRI) of the whole compartment with adjacent joints. CT scan is recommended only in the case of diagnostic problems or doubt, to provide clearer visualization of calcification, periosteal bone formation, or cortical destruction. [28]

Both guidelines agree that biopsy is required to confirm the diagnosis prior to any surgical procedure and should be performed at a specialized center that will provide the definitive treatment. [27, 28]

Classification

ESMO guidelines recommend specifying the tumor type and subtype according to the 2013 World Health Organization (WHO) classification. [28] Under the WHO classification system, tumors are further classified as benign, intermediate, or malignant. Bone sarcomas are classified by group (eg, chondrogenic, osteogenic, fibrohistiocytic, Ewing sarcoma) and further subtyped within each group. [29]

NCCN guidelines recommend that the final pathologic evaluation include assessment of surgical margins as well as the size/dimensions of tumors. [27]

With patients who present with locally advanced or metastatic chondrosarcoma, chordoma, or osteosarcoma, physicians should discuss the options surrounding deep-sequencing genomic tests, which may identify mutations that may be responsive to specific therapies and thus may guide referral to clinical trials. Use of these tests is not part of current NCCN guidelines but is increasingly part of oncologic practice. A further barrier is that even when available, these tests are typically not reimbursed by private or government insurance.

Grading and Staging Systems

A number of staging systems are used for bone tumors. The ESMO guidelines do not provide a specific recommendation for which system should be followed. [28]  NCCN follows both the tumor-node-metastasis (TNM) classification of the American Joint Cancer Committee/Union for International Cancer Control (AJCC/UICC) [30] and the Surgical Staging System from the Musculoskeletal Tumor Society (MTS) [31] for staging. [27]

Treatment

NCCN recommendations for treatment of chordoma are as follows [27] :

  • Enrollment in a clinical trial should be considered when available; in addition, whenever possible, patients should be referred to a tertiary care center with expertise in sarcoma, for treatment by a multidisciplinary team.

  • Wide excision with or without radiation therapy for tumors of the sacrum and mobile spine.

  • Intralesional excision with or without radiation therapy is the preferred treatment for resectable skull base tumors; re-resection can be considered with positive surgical margins; postoperative radiation for improved local control.

  • Adjuvant radiation therapy can be considered for large extracompartmental tumors or for positive surgical margins after resection.

  • Radiation therapy is the primary treatment for unresectable tumors regardless of location.

  • Dedifferentiated chordomas are treated according to soft tissue sarcoma management guidelines.

  • For local recurrence, surgical excision with or without radiation therapy and/or chemotherapy.

  • For metastatic disease, options include chemotherapy and/or surgical excision and/or radiation therapy and/or best supportive care.

  • ESMO guidelines are in general agreement with the NCCN guidelines. [28]