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一例儿童软骨黏液样纤维瘤样骨肉瘤病例。

A case of a paediatric chondromyxoid fibroma-like osteosarcoma.

作者信息

Boumeriem Khaoula, Bourekba Iliass, Allali Nazik, Chat Latifa, El Haddad Siham

机构信息

Department of Radiology, Children's Hospital of Rabat, Rabat MA 10000, Morocco.

出版信息

BJR Case Rep. 2025 Mar 1;11(2):uaaf011. doi: 10.1093/bjrcr/uaaf011. eCollection 2025 Mar.

Abstract

Chondromyxoid fibroma-like osteosarcoma (CMF-OS) is an exceptionally rare and low-grade variant of osteosarcoma, as classified by the World Health Organization. Misdiagnosis is common in CMF-OS, often leading to delays in definitive surgical intervention. CMF-OS exhibits variable imaging features, frequently mimicking chondromyxoid fibroma. It may present as osteolytic, osteogenic, or expansive lesions, often associated with soft tissue invasion, cortical disruption, and occasionally a periosteal reaction. Cases have been reported in diverse anatomical locations, including the craniofacial region and bones of the lower limbs. Histologically, CMF-OS is distinguished by its unique mucoid appearance, characterized by loose aggregates of stellate and spindle-shaped tumour cells embedded within a highly myxoid stroma. Surgical resection remains the cornerstone of treatment for CMF-OS, emphasizing the importance of accurate diagnosis to facilitate timely and appropriate management.

摘要

软骨黏液样纤维瘤样骨肉瘤(CMF-OS)是世界卫生组织分类的一种极为罕见的低级别骨肉瘤变体。CMF-OS的误诊很常见,常常导致确定性手术干预的延迟。CMF-OS具有多样的影像学特征,常类似软骨黏液样纤维瘤。它可能表现为溶骨性、成骨性或膨胀性病变,常伴有软组织侵犯、皮质破坏,偶尔还有骨膜反应。已报道的病例分布于不同解剖部位,包括颅面部区域和下肢骨骼。在组织学上,CMF-OS以其独特的黏液样外观为特征,表现为星状和梭形肿瘤细胞松散聚集于高度黏液样基质中。手术切除仍然是CMF-OS治疗的基石,强调准确诊断对于促进及时和恰当管理的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eab/11922549/267077a7e179/uaaf011f1.jpg

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