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

Abstract and Introduction

Abstract

After initial treatment of sarcoma, disease progression may occur in the form of local recurrence, pulmonary metastases, or extrapulmonary metastases. As such, surveillance is an important aspect of management, but no universally accepted practice standards are found. In the absence of strong evidence, and to allow for individualized care, existing guidelines contain flexibility in terms of both the frequency and modality of surveillance. In general, they agree that follow-up should be more intense in the early years after treatment, especially for high-grade sarcomas, and continue for at least 10 years. For local recurrence, data suggest that physical examination is usually sufficient for monitoring; in addition, some guidelines endorse imaging routinely, whereas others only as clinically indicated. For pulmonary metastasis, either radiograph or CT is recommended, with the latter having theoretical advantages but no proven survival benefit to date. Extrapulmonary metastases are rare in most sarcoma types, so the literature only supports extrapulmonary surveillance for certain diagnoses. This topic is complicated by the diversity of sarcomas, the limited evidence, and the indefinite, often conflicting recommendations; therefore, it is critical for providers to understand the existing research and guidelines to determine optimal surveillance strategies for their patients.

Introduction

Sarcomas are a diverse group of over 50 malignancies of mesenchymal origin. They arise as primary tumors of the musculoskeletal system and affect patients of all ages. The incidence of sarcoma in the United States was estimated at 15,000 cases in 2014, and although it has been increasingly diagnosed in recent decades, it remains a relatively rare disease. Five-year mortality for sarcoma in general is approximately 35% but varies greatly based on the disease type; most low-grade tumors have minimal risk, whereas some high-grade tumors are almost universally fatal.

Primary, nonmetastatic sarcomas are usually managed with some combination of chemotherapy, radiation therapy, and surgical resection. Although these initial treatments are curative in many cases, disease progression can occur in the form of local recurrence (LR) and/or distant metastases. LR is problematic because it can cause symptoms and require additional, often more complex surgical procedures. Metastatic disease, which can develop in the presence or absence of LR, is the major cause of mortality associated with sarcoma. Metastases most commonly affect the lungs but can also occur in other viscera, soft tissues, or bones. Detecting disease progression early has the potential to reduce morbidity and mortality in certain cases, making surveillance an important aspect of management.

Despite this fact, the optimal imaging modality and frequency for surveillance remains uncertain. The rarity of sarcomas, combined with the broad range of disease types and patients affected, makes this a challenging cohort to study and treat. Higher frequency surveillance with more advanced imaging techniques may detect disease recurrence sooner and, thus, facilitate treatment. Conversely, disadvantages of more intense surveillance include increased time for physicians and patients, radiation exposure, and risk of false-positive results requiring costly and unnecessary additional workup. Resource utilization is also a notable consideration, as the expense associated with surveillance can vary dramatically with frequency and modality; for example, a review in 2004 describes a total of 54 different surveillance strategies for soft-tissue sarcomas (STS) with charges ranging from $485 to $21,235, a 42.8-fold cost differential.[1]

Owing to the complexity of this topic, no universal practice standards are found with respect to surveillance for LR, pulmonary metastases (PMs), or extrapulmonary metastases (EMs). The existing guidelines contain flexibility in both the recommended frequency and modality of surveillance; physicians must therefore understand the options to determine how to follow-up their patients. The purpose of this article is to review the available evidence and consensus-based guidelines on sarcoma surveillance to help inform these decisions.

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