Osteosarcoma Guidelines

Updated: Sep 12, 2016
  • Author: Charles T Mehlman, DO, MPH; Chief Editor: Harris Gellman, MD  more...
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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 osteosarcoma have been published by the following organizations:

  • National Comprehensive Cancer Network (NCCN) [39]
  • European Society for Medical Oncology (ESMO) [40]

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 aged 40 years or older, the recommended workup includes the following:

  • 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. [39]

European Society for Medical Oncology (ESMO) guidelines recommend followup of an abnormal radiograph with magnetic resonance imaging (MRI) of the whole compartment with adjacent joints. CT is recommended only in the case of diagnostic problems or doubt, to provide clearer visualization of calcification, periosteal bone formation, or cortical destruction. [40]

The two 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. [39, 40]

Classification

ESMO guidelines recommend specifying the tumor type and subtype according to the 2013 World Health Organization (WHO) classification. [40]  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. The most common pathologic subtype is conventional central osteosarcoma. [41]

NCCN guidelines recommend that the final pathologic evaluation include assessment of surgical margins as well as the size/dimensions of tumors. [39]

With patients who present with locally advanced or metastatic 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. [40] NCCN follows both the tumor-node-metastasis (TNM) classification of the American Joint Cancer Committee/Union for International Cancer Control (AJCC/UICC)  [42] and the Surgical Staging System from the Musculoskeletal Tumor Society (MTS) [43] for staging. [39]

Treatment

Recommendations for the treatment of osteosarcoma from NCCN and ESMO vary by disease stage. [39, 40]

Treatment recommendations for stages IA-IB (low grade) osteosarcomas are as follows:

  • 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
  • Localized, low-grade osteosarcomas – The NCCN recommends wide excision alone; chemotherapy prior to excision is not typically recommended but could be considered for periosteal lesions [39]
  • Low-grade intramedullary and surface osteosarcoma and periosteal sarcomas with pathological findings of high-grade disease – The NCCN recommends postoperative chemotherapy [39] ; the ESMO recommends surgery alone for these tumors and finds no benefit for chemotherapy for periosteal lesions [40]
  • Unresectable or incompletely resected osteosarcoma – The NCCN and ESMO guidelines concur that combined photon/proton or proton beam radiotherapy for local control is an option [39, 40]

NCCN treatment recommendations for stages IIA-IVB (high grade) and metastatic disease include the following [39] :

  • 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
  • Preoperative chemotherapy is recommended for all stages of high-grade disease (category 1)
  • If good margins can be achieved, limb-sparing surgery is preferred for patients with good histologic response to chemotherapy; amputation for tumors in unfavorable anatomical locations
  • Postoperative chemotherapy should continue with preoperative regimen if there has been a good histologic response; for patients with a poor response, consider postoperative chemotherapy with a different regimen
  • Surgical re-resection with or without radiation therapy for positive margins should be considered
  • For unresectable osteosarcomas following preoperative chemotherapy, consider radiation therapy or chemotherapy

NCCN treatment recommendations for metastatic disease at presentation include the following:

  • 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
  • For resectable metastatic disease (pulmonary, visceral or skeletal), preoperative chemotherapy followed by wide excision of primary tumor; chemotherapy and metastasectomy is also a treatment option
  • For unresectable metastatic disease, chemotherapy with or without radiation therapy; reassess primary site for local control
  • ESMO recommends that primary metastatic osteosarcoma be treated with a curative intent, following the principles of non-metastatic osteosarcomas [40]

For relapsed or refractory disease, NCCN guidelines recommend second-line chemotherapy, resection, or both. Options for disease progression after second-line therapy include the following [39] :

  • Re-resection, if possible
  • Clinical trial
  • Samarium-153 ethylene diamine tetramethylene phosphonate (SM-EDTMP)
  • Palliative radiation therapy or best supportive treatment
  • ESMO guidelines note that there is no accepted standard regimen for second-line chemotherapy for recurrent disease, but ifosfamide with or without etoposide with or without carboplatin, or gemcitabine and docetaxel or sorafenib may be considered. Radiation therapy, including samarium, may be used for palliation. [40]