Atypical cartilaginous tumors: Diagnosis and therapy

Abstract

Background: Atypical cartilagineous tumours (ACTs) of short and long bones, previously termed as chondrosarcoma G1, are of locally aggressive behavior, but have little metastatic potential. Differentiation between ACTs and enchondromas is complex on clinical, radiological and histopathological basis. Objectives: Epidemiology, diagnosis, and therapy of ACTs with specific focus on distinction from enchondroma. Materials and Methods: Summary of international literature on ACTs and enchondromas. Results: The incidence of enchondromas, and even more the one of ACTs, has increased over the years, indicating more frequent application of diagnostics. Contrary to enchondroma, ACTs may be associated with pain and present with radiological signs indicative of aggressive growth, such as deep endosteal scalloping. Performing biopsy only to differentiate between suspected enchondroma and ACT may be unhelpful as the acquired tissue specimen can lead to a “sampling error”. Definite surgical therapy of ACTs of long and short bones has changed over the last years, shifting from radical tumour resection towards intralesional curettage. A primary watch-and-wait approach is possible in enchondroma using regular MRI check-ups. Conclusions: Other than enchondroma, ACTs present with radiologic signs of aggressive growth. Nowadays, ACTs are preferably treated by intralesional curettage. Diagnosis, therapy and follow-up of cartilaginous tumours should be carried out at specialized tumour centers.