Aim: Follow-up strategies for primary extremity soft-tissue sarcomas (eSTS) in adults were evaluated in a systematic review of the published literature. Material and Methods: The published literature was reviewed using PubMed. Of 136,646 studies published between 1985 and 2019, 78 original articles met the inclusion criteria. Articles were selected on the basis of the PRISMA guidelines. The selected articles were then cross-searched to identify further publications. August 1, 2019 was used as the concluding date of publication. Results: A variety of follow-up schedules have been reported in recently published literature. Two official guidelines have been approved by international societies. The guidelines distinguish between high-and low-grade STS, but mention a wide range of follow-up intervals. Established tools of follow-up include computed tomograph, X-rays of the chest, and magnetic resonance imaging of the primary tumor site in addition to clinical observation and physical examination. Conclusion: Further research will be needed to establish evidence-based guidelines and schedules for follow-up strategies in patients with eSTS.Soft-tissue sarcomas (STS) of the extremities constitute less than 1% of all malignant tumors (1-4). Patients with highgrade STS are at risk of developing local recurrence (LR) and distant metastases (DM) after having undergone successful surgical resection of the primary tumor (5-10). Rates of STS differ in terms of size, grade, and subtype (5, 11). According to the published literature, 12,750 new cases of STS and 5,270 deaths occurred in the United States, resulting in a mortality rate of about 40% in 2019 (12)(13)(14). Recent published studies have revealed a yearly incidence of about 4-5/100,000 in Europe; liposarcoma and leiomyosarcoma are the most common histological subtypes (15-17). Nearly every third patient with primarily local STS will develop DM during the follow-up period, most likely in the lungs (18).The large majority of STS are primarily located in the extremities; about 40% occur in the lower limbs (1,2,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30). The second most frequent location is the abdomen (retroperitoneal or visceral); the lesions are usually very voluminous at the time of presentation (1,(19)(20)(21)(22)(23). More than 75% of malignant STS are located beneath the fascia (20,22,23,27). The median age of patients at the initial diagnosis of primary STS is around 50 years and a slight preponderance of the male gender has been reported (1,4, 21,22,25,26,28,30,31).STS are divided into more than 50 histological subtypes, arising from mesodermal or neuroectodermal tissue (15, 19,32). The histological classification is based on the differentiation of tumor cells, regardless of their origin (33). The European Society of Medical Oncology (ESMO) (21, 34), as well as 34) have identified more than 80 histological entities that may be further subdivided into even greater numbers of subsets. The National Comprehensive Cancer Network (NCCN) makes a rough division of...