背景:软骨粘液样纤维瘤样骨肉瘤(CMF-OS)是一种极其罕见的低级别中央骨肉瘤(LGCO)亚型,占病例的10%,难以诊断。CMF-OS在放射学检查和活检中经常被误诊,即使在最初的手术之后。由于其低级分类和实际的高级行为,其处理是一个有争议的问题。
方法:我们回顾性回顾了2008年至2019年间2000多名骨肉瘤患者的病历;确定了11例CMF-OS患者,其中六名患者由我们的机构治疗,具有完整的临床特征,包括治疗和预后,回顾了放射学和病理学特征。经病理证实,三名男性和三名女性的中位年龄为46岁(22-56岁)。CMF-OS的放射学表现是可变的,因此放射学误诊是常见的。然而,不能忽视恶性放射学外观.赋予CMF-OS诊断的最独特的病理特征是在软骨粘液样纤维瘤(CMF)样背景下,肿瘤细胞直接产生类骨质。基于CMF的综合数据进行鉴别诊断,LGCO,软骨肉瘤(CHS),常规骨肉瘤(COS),等。,是需要的。所有患者均接受手术和化疗,一名患者接受了额外的放疗。然而,复发和转移在CMF-OS患者中很常见。CMF-OS的相对侵袭性生物学行为与该疾病的低度分类相反。
结论:识别CMF-OS并将其与CMF区分开来很重要,CHS,COS和其他LGCO。尽管CMF-OS的分级较低,但其预后相对较差。
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.
METHODS: 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.