Abstract

Simple SummaryOver the last decade the incidence of enchondroma and atypical cartilaginous bone tumors (ACTs) increased enormously. Management of these tumors in the long bones is shifting towards active surveillance, as negative side effects of surgical treatment seem to outweigh the potential benefits. To support development of evidence-based guidelines for active surveillance, we studied the natural course of enchondroma and ACTs in the long bones. In this study, MRI analysis of 128 cases was performed with a minimum interval of 24 months between baseline and last MRI. Our data showed that the majority of the cartilaginous tumors (87%) remained stable or showed regression on MRI. Only 13% showed some progression on MRI, although none of the tumors developed characteristics of high-grade chondrosarcoma. Based on our results, active surveillance is considered safe for enchondroma and ACTs of the long bones, and follow-up schemes should be tailored on natural course.Management of atypical cartilaginous tumors (ACTs) in the long bones is shifting towards active surveillance to avoid unnecessary surgeries. The frequency and duration of active surveillance for these tumors is unclear as there is little knowledge of its biological behavior. In this retrospective study, we examined the natural course of enchondroma and ACTs through active surveillance. A total of 128 central cartilaginous tumors, located in the long bones, with a minimum interval of 24 months between baseline and last MRI were included. MRI characteristics (e.g., size, scalloping, fat entrapment) were scored and tumors were classified according to the changes between MRIs. Mean follow-up of this study was 50 months, range = 25–138 months. The majority of the cartilaginous tumors (87%) remained stable (n = 65) or showed regression (n = 46) on MRI. A total of 87% of the cases that developed tumor regression presented with entrapped fat at diagnosis. Only 13% (n = 17) showed some progression on MRI, although none of the tumors developed characteristics of high-grade chondrosarcoma. Based on our results, active surveillance is considered safe for enchondroma and ACTs of the long bones. We propose active surveillance for all asymptomatic enchondroma or ACTs in the long bones irrespective of tumor size, and follow-up schemes should be tailored on natural course.

Highlights

  • Nowadays central cartilaginous bone tumors are reported as the most common primary bone tumor

  • None of the cases included in this study showed any aggressive magnetic resonance imaging (MRI) characteristics often seen in high-grade chondrosarcoma (HGCS) during follow-up

  • To support development of evidence-based guidelines for active surveillance, we studied the natural course of enchondroma and atypical cartilaginous tumors (ACTs) in the long bones

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Summary

Introduction

Nowadays central cartilaginous bone tumors are reported as the most common primary bone tumor. Enchondroma are benign cartilaginous tumors, whereas chondrosarcoma are malignant cartilaginous tumors. Chondrosarcomas are divided into grades ranging from 1 to 3 which correlate with aggressiveness and; with local recurrence rate, metastatic potential and the disease specific potential. In the latest 2020 WHO classification, a clear distinction between CS1 in the axial skeleton and appendicular skeleton was made [2]. Tumors in the axial skeleton are still termed CS1, reflecting the more aggressive local behavior and poorer clinical outcome of tumors at these sites, whereas CS1 in the appendicular skeleton are termed atypical cartilaginous tumors (ACTs). Due to the numerous similarities of enchondroma and ACTs on imaging, differentiating these tumors is problematic [3,4,5]. High-grade chondrosarcoma is recognized by aggressive radiologic features such as bone destruction and soft tissue extension

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