☆ 4.6 Article

Periosteal chondrosarcoma: A case series in a referral center with survivorship analysis

EJSO (2022)

Journal

EJSO

Volume 48, Issue 8, Pages 1730-1738

Publisher

ELSEVIER SCI LTD

DOI: 10.1016/j.ejso.2022.05.024

Keywords

Periosteal; Chondrosarcoma; IDH; Prognosis

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Abstract

This study retrospectively analyzed 55 cases of periosteal chondrosarcomas and found that they exhibit a low-grade behavior that can be adequately treated with marginal excisions. Clinical and morphologic parameters do not seem to predict their outcome.

Background: Periosteal chondrosarcomas are among the rarest types of chondrosarcomas dealt with in few small series of cases. In this study, we aimed to present our experience with this chondrosarcoma, seek for prognostic factors for OS and DFS and survey the status of IDH1 and IDH2.Results: 55 periosteal chondrosarcomas were retrospectively identified. Median age was 37 years, there was a male predominance (62%). The great majority of cases involved the metaphysis of long bones of the extremities. The median size of the tumors was 7.5 cm. Thirty patients underwent to subtotal surgical resection, 22 to tangential resection and the remaining 3 to amputation. The margins, reported in 54 cases, were wide/radical in 38 patients (70.4%), marginal in 9 (16.7%) and intralesional in 7 (12.9%). Histologically, 23 (42%) were grade 1; 27 (49%), grade 2; 3 (5%), grade 3 and 2 (4%) were dedifferentiated. A third of cases in which mutational analysis was feasible harbored heterozygous mutations in codon 132 of IDH1. Fifty-four cases were included for follow-up (median, 137 months). Four patients had local recurrences and six patients developed metastasis to the lungs. All patients that developed metastasis died of disease, two died of unrelated causes and 46 were alive without disease. OS and DFS was not found to be statistically associated with clinical and pathological parameters considered.Conclusions: periosteal chondrosarcomas exhibit a low-grade behavior that can be adequately treated with marginal excisions. Clinical and morphologic parameters do not seem to predict their outcome. (c) 2022 Elsevier Ltd, BASO - The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.

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IDH Mutations in Chondrosarcoma: Case Closed or Not?

Sanne Venneker, Judith V. M. G. Bovee

Summary: Chondrosarcomas frequently carry isocitrate dehydrogenase (IDH) gene mutations and these mutations play a significant role in early stages of cartilage tumour development. However, their role in later stages remains unclear. Other types of IDH mutant tumours have shown the impact of this mutation on patient outcomes and therapies. Factors such as tumour type, elevated oncometabolite levels, the type of model used in preclinical studies, and additional (epi)genetic alterations could explain the discrepancy in the role of IDH mutations. Recent studies have identified subgroups within IDH wildtype and mutant chondrosarcomas, which should be further studied to improve treatment and predictive biomarkers.

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