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Published in: BMC Musculoskeletal Disorders 1/2020

01-12-2020 | osteosarcoma | Case report

Chondromyxoid fibroma-like osteosarcoma: a case series and literature review

Authors: Jingyu Zhong, Liping Si, Jia Geng, Yue Xing, Yangfan Hu, Qiong Jiao, Huizhen Zhang, Weiwu Yao

Published in: BMC Musculoskeletal Disorders | Issue 1/2020

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Abstract

Background

Chondromyxoid fibroma-like osteosarcoma (CMF-OS) is an exceedingly rare subtype of low-grade central osteosarcoma (LGCO), accounting for up to 10% of cases and making it difficult to diagnose. CMF-OS is frequently misdiagnosed on a radiological examination and biopsy, even after the initial operation. Its treatment is a controversial issue due to its low-grade classification and actual high-grade behavior.

Case presentation

We retrospectively reviewed the medical charts of more than 2000 osteosarcoma patients between 2008 and 2019; 11 patients with CMF-OS were identified, of which six patients were treated by our institution with complete clinical characteristics, including treatment and prognosis, radiological and pathological features were reviewed. Three males and three females with a median age of 46 (range 22–56) years were pathologically proven to have CMF-OS. The radiological presentation of CMF-OS is variable, thus radiological misdiagnoses are common. However, one must not ignore a malignant radiologic appearance. The most distinctive pathological feature conferring the diagnosis of CMF-OS is the presence of osteoid production directly by the tumor cells under a chondromyxoid fibroma (CMF)-like background. Differential diagnoses based on comprehensive data from CMF, LGCO, chondrosarcoma (CHS), conventional osteosarcoma (COS), etc., are needed. All patients were treated with an operation and chemotherapy, and one patient received additional radiotherapy. Nevertheless, recurrence and metastasis are common in CMF-OS patients. Relatively invasive biological behavior of CMF-OS is against the low-grade classification of this disease.

Conclusions

It is important to recognize CMF-OS and distinguish it from CMF, CHS, COS and other LGCOs. CMF-OS has a relatively poor prognosis despite its low-grade classification.
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Metadata
Title
Chondromyxoid fibroma-like osteosarcoma: a case series and literature review
Authors
Jingyu Zhong
Liping Si
Jia Geng
Yue Xing
Yangfan Hu
Qiong Jiao
Huizhen Zhang
Weiwu Yao
Publication date
01-12-2020
Publisher
BioMed Central
Published in
BMC Musculoskeletal Disorders / Issue 1/2020
Electronic ISSN: 1471-2474
DOI
https://doi.org/10.1186/s12891-020-3063-5

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