骨和累及骨的外周性原始神经外胚层瘤的临床病理与影像学表现

Clinical,Pathological and Imaging Features of Peripheral Primitive Neuroectodermal Tumors of the Bone and Involving Bone

目的探讨骨和累及骨的外周性原始神经外胚层瘤(pPNETs)的临床病理与影像学表现。资料与方法7例中6例有X线检查、2例有CT检查、4例有MR检查,分析其临床病理和影像学特点。结果6例X线片上均呈溶骨性骨质破坏,其中2例病变区存在骨质硬化,5例合并软组织肿块,均未见骨膜反应。2例CT像上均为溶骨性骨质破坏合并软组织肿块形成,软组织内未见钙化或骨化。MRI检查4例中有3例病变在T1WI呈中等信号,1例在T1WI呈低信号,4例在T2WI上均呈中、高信号,信号不均匀,增强扫描呈中度不均匀强化。4例均合并软组织肿块,其中3例有囊变坏死区。结论影像学上骨内病变呈溶骨性破坏,可伴有病变区的骨质硬化而一般无骨膜反应,并伴有较大软组织肿块者应考虑到pPNETs的可能。

Objective To analyze the clinical, pathological and imaging features of peripheral primitive neuroectederreal tumors （pPNETs） of the bone and involved bone. Materials and Methods 7 cases were included. X-ray plain film, CT and MRI were performed in 6, 2 and 4 cases, respectively. Their clinical, pathological and imaging data were reviewed. Results The X-ray findings of 6 cases were lytic bone destruction, bone sclerosis （ 2 cases）, and soft tissue mass （5 cases）. No case had periosteal reaction. CT manifestations were consisted of lytic bone destruction with soft tissues masses in 2 cases. No calcification or ossification could be seen in the masses. Isointensity （3 cases） and hypointensity （1 case） could he shown on T1W images and heterogeneous isohyperintensity（4 cases） on T2W images. The lesions were enhanced intermediately on enhanced T1 W images. Soft tissue masses were found in all 4 cases, and cystic regions and necrosis were demonstrated in 3 cases. Conclusion pPNETs should be considered when imaging findings include lytic bone destruction without periosteal reaction on bone combined with a large soft tissue mass.