儿童侵袭性骨巨细胞瘤1例并文献复习

One Case of Invasive Giant Cell Tumor of Bone in a Child and Literature Review

目的探讨儿童侵袭性骨巨细胞瘤的临床病理学特征、诊断和鉴别诊断。方法研究泉州市正骨医院诊断的一例儿童侵袭性骨巨细胞瘤的临床资料、病理形态学特征、免疫组化结果,并结合相关文献进行讨论。结果女性患儿,9岁4个月,外伤后影像学检查左胫骨近端肿瘤前侧皮质不连续,软组织密度影突出骨外,病灶向上跨越骺板,累及骨骺。术后送检肿物镜下见梭形、短梭形单核肿瘤细胞和破骨细胞样多核巨细胞构成,未见肿瘤性成骨和软骨样基质,肿瘤侵蚀破坏宿主骨小梁并穿透骨皮质到骨膜组织。免疫组化:Vimentin(+)、H3F3A G34W(+)、P63单核细胞(+)、CD68多核巨细胞(+)、CD34(-)、SMA(-)、S100(-)、H3.3 K36M(-)、Bcl-2(-)、Ki-67约25%阳性。结论儿童侵袭性骨巨细胞瘤是一种罕见的富于巨细胞的破骨细胞肿瘤,依据影像学、形态学和免疫组化及结合基因检测可明确诊断。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of invasive giant cell tumor of bone in children.Methods The clinical data,histopathological features and immunohistochemical results of a child invasive giant cell tumor of bone diagnosed in Quanzhou Orthopedic-traumatological Hospita were studied.Results The female child,aged 9 years and 4 months,showed a discontinuous anterior cortex of the tumor at the proximal left tibia with soft tissue density protruding outside the bone.The lesion crossed the epiphysis plate upward and involved the epiphysis.The objective of postoperative examination showed spindle and short spindle mononuclear tumor cells and osteoclast like multinucleated giant cells,but no neoplastic osteoblasts and chondroid matrix were found.The tumor eroded and destroyed the trabecular bone of the host and penetrated the bone cortex to the periosteum tissue.Immunohistochemistry:Vimentin(+),H3F3A G34W(+),P63 monocytes(+),CD68 multinucleated giant cells(+),CD34(-),SMA(-),S100(-),H3.3 K36M(-),Bcl-2(-),about 25%positive for Ki-67.Conclusion Invasive giant cell tumor of bone in children is a rare osteoclast tumor rich in giant cells.