What are the treatment guidelines for chordoma?

Updated: Jul 01, 2020
  • Author: Mrinal M Gounder, MD; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

Answer

NCCN recommendations for treatment of chordoma are as follows [1] :

  • Enrollment in a clinical trial should be considered whenever 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 may be the best feasible treatment for resectable skull base tumors when wide excision is not possible; re-resection can be considered with positive surgical margins; postoperative radiation for improved local control

  • Adjuvant radiation therapy can be considered for large 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 and chemotherapy can be used where clinincally indicated

  • 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-PaedCan-EURACAN guidelines are in general agreement with the NCCN guidelines. [2]


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