Sarcoma Surveillance: A Review of Current Evidence and Guidelines

Cara A. Cipriano, MD, MS; Eugene Jang, MD, MS; Wakenda Tyler, MD, MPH

Disclosures

J Am Acad Orthop Surg. 2020;28(4):145-156. 

In This Article

Review of Guidelines

Current guidelines for sarcoma surveillance have been issued by organizations including the National Comprehensive Cancer Network (NCCN), the European Society for Medical Oncology (ESMO), the British Sarcoma Group (BSG), and the MSTS in conjunction with the American Academy of Orthopaedic Surgeons (MSTS/AAOS). In this section, we will review these guidelines for both soft-tissue and bone sarcomas and the levels of evidence on which they are based.

National Comprehensive Cancer Network

The 2018 NCCN guidelines for STS management and surveillance[42] are based on level 2A evidence, and the authors disclose that "very limited data are available in the literature on effective surveillance strategies" for STS (Table 1). The NCCN emphasizes risk-stratification for surveillance, with increased attention to patients with larger, high-grade tumors in the early postoperative years. For LR surveillance, they state that physical examination may suffice in some situations, and ultrasonography may be useful for superficial tumors when performed by an experienced technician. In all other cases, the guidelines generally recommend periodic MRI with and without contrast or CT with contrast based on the risk of disease progression. For chest imaging, the NCCN prefers CT over radiograph but acknowledges that no study has demonstrated improved outcomes with CT surveillance.

The NCCN guidelines for bone sarcoma surveillance were published in 2013 and revised in 2017.[43,44] Similar to the soft-tissue guidelines, they are based on relatively low-level evidence supported by expert consensus. Because of the known variation in treatment modalities and patterns of recurrence between specific diagnoses, the guidelines were categorized based on the four most common bone tumors encountered (chondrosarcoma, osteosarcoma, chordoma, and Ewing's sarcoma) and giant cell tumor of bone, a locally aggressive tumor that is considered benign but has metastatic potential (Table 1).[43,44]

European Society for Medical Oncology

In the 2018 ESMO STS guidelines,[45] the authors acknowledge that few published data indicate optimal guidelines for follow-up of patients with STS and offer a range of acceptable options as practical guidelines (Table 2). Of note, they state that use of sensitive advanced imaging modalities (ie, MRI and CT) may diagnose LR and PM earlier; however, "it has not been demonstrated that this is beneficial, or cost effective, compared with the clinical assessment of the primary site and regular chest X-rays."[45]

For bone sarcomas, the most recent ESMO guidelines (2014)[46] again disclose that no clear consensus exists among the expert panel or in the literature to support one definite practice before providing some general guidelines. Instead of specific recommendations regarding intervals or modality, they allow for variability depending on local practice and other tumor- or patient-related risk factors (Table 2). Although they suggest decreasing frequency of follow-up over time, they do not specify an end point given the potential for LR, metastases, and functional deficits may occur >10 years after diagnosis.

British Sarcoma Group

In 2010 and 2016, the BSG published recommendations for treatment and follow-up of soft-tissue and bone sarcomas based on expert opinion and data collected from various sources, including the NCCN and ESMO. In the same manner as these other organizations, they point out the limitations of current evidence on sarcoma surveillance, and as such, their guidelines are designed to be general rather than prescriptive[47,48] (Table 3). Of note, the group defines a standard follow-up visit as including "(1) review of any new symptoms reported by the patient, (2) clinical examination to focus on LR, with imaging follow-up where indicated by clinical suspicion, (3) routine chest radiograph to exclude pulmonary metastases, and (4) monitoring for late effects of treatment."[47]

For STS, the BSG recommends physical examination as the basis of LR surveillance, with advanced imaging based on clinical indications. They suggest that chest imaging be reserved for patients who would receive intervention if metastatic disease was detected early.[47] In addition, of note, they advocate for the use of chest radiograph rather than CT for PM surveillance. They recommend clinical and chest follow-up more frequently for high- than low-grade tumors, although both groups should be followed up for 10 years because of the risk of late recurrences (Table 3).

BSG guidelines for bone sarcomas are similar to those for STS[48] (Table 3). The authors indicated that strong recommendations for local imaging could not be made and that this should be based on clinical indications. In addition, given recent evidence that patients often identify their own recurrences, the BSG surveillance plans include patient education on self-examination and appropriate measures to take if symptoms suggest LR. Finally, owing to the risk of late disease progression and other long-term treatment complications, the group likewise could not recommend a definitive end point for surveillance.[48]

Musculoskeletal Tumor Society and American Academy of Orthopaedic Surgeons

In two recent survey studies of MSTS members, >90% of respondents expressed concerns about the lack of evidence-based guidelines for sarcoma surveillance.[29,49] In 2017, the MSTS identified this as the organization's single highest research priority and subsequently, in conjunction with the AAOS, defined appropriate use criteria to aid in clinical decision making in this area.

The "surveillance of local recurrence and distant metastases after surgical treatment of bone and soft-tissue sarcomas" criteria were developed by a panel of experts representing orthopaedic oncology, surgical oncology, medical oncology, and musculoskeletal radiology. The process was initially informed by a systemic review of the literature on the subject. A writing subset of the group determined that the most important criteria were risk of metastatic disease, risk of LR, tumor tissue of origin, extrapulmonary metastatic risk, history of recurrence, and time since treatment. They developed 144 scenarios with various combinations of these factors, as well as 27 treatment options. The remainder of the panel reviewed all scenarios and voted on the most appropriate imaging surveillance modalities for each, after which the entire group met to discuss areas of discrepancy. The independent voting process was then repeated, and guidelines were formulated based on the results, with the strength of the recommendation correlating with the degree of expert consensus. The final document was approved by the leadership of the MSTS and AAOS and was incorporated into AAOS OrthoGuidelines in 2018.

This decision-making aid can be accessed through the AAOS OrthoGuidelines website under the Appropriate Use Criteria tab[50] or on a mobile device through the OrthoGuidelines application, available for iOS and Android.[51] When healthcare providers enter characteristics of a specific patient scenario, the program lists surveillance plans (including frequency and modality) that are "appropriate," "may be appropriate," and "rarely appropriate" in that context. Like the previously discussed guidelines, this tool offers a range of options for surveillance, allowing providers to exercise clinical judgment in the absence of strong evidence.

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