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目的探讨骨软化或佝偻病相关的间叶组织肿瘤的临床病理特点。方法回顾分析10例患者的临床资料,观察10例骨软化相关的肿瘤组织的形态和免疫表型[免疫组织化学SP法染色,所用抗体包括波形蛋白、S100、平滑肌肌动蛋白(SMA)、结蛋白、CD34、AE1/AE3、Ki67、HMB45]。结果患者男性6例,女性4例,年龄范围28～69岁(平均45.6岁);患者均有2～27年(平均9.6年)骨痛、关节痛和活动困难的病史,检查发现低血磷、高尿磷;肿瘤最大径1～7cm不等(平均3.5cm);瘤组织为间叶组织来源(仅2例颌骨病变中见不明显的条索状上皮),可见多少不等的梭形纤维母细胞样细胞、脂肪细胞、软骨样细胞、黏液样细胞等,瘤组织富于血管,8例病变中有少见的絮状或不规则砂砾样钙盐沉积,2例发生于软组织的肿瘤周边见骨壳形成,3例组织中可见非尿酸盐结晶;9例细胞分裂象少见,1例核分裂象多见并且异型性明显;瘤细胞波形蛋白阳性,5例SMA部分阳性,3例CD34部分瘤细胞阳性,结蛋白、S100、AE1/AE3均阴性,Ki67指数(8例≤4%,仅1例为30%);AB/PAS染色:8例肿瘤黏液基质和血管周围黏液样变呈AB染色阳性。结论骨软化相关的肿瘤多为良性或低度恶性的间叶组织肿瘤,因组织学具有多样性而易误诊,掌握其共性特征并结合临床资料方能正确诊断。
Objective To investigate the clinicopathological features of osteomalacia or mesenchymal tumor associated with rickets. Methods The clinical data of 10 patients were retrospectively analyzed. Morphology and immunophenotype of 10 cases of osteomalacia were observed. Immunohistochemical SP staining was used. Antibodies used included vimentin, S100, SMA, Protein, CD34, AE1 / AE3, Ki67, HMB45]. Results There were 6 males and 4 females, ranging in age from 28 to 69 years (average 45.6 years). The patients had a history of pain, joint pain and difficulty in activity for 2 to 27 years (average 9.6 years). Hypophosphatemia , High urinary phosphate; the largest diameter of tumor ranging from 1 to 7cm (average 3.5cm); mesenchymal tissue source for mesenchymal tissue (only 2 cases of jaw lesions were not obvious in the cable epithelium), showing how many different shuttle Shaped fibroblast-like cells, adipocytes, cartilage-like cells, myxoid cells, etc. The tumor tissue is rich in blood vessels. There are rare flocculent or irregular gravel-like calcium deposits in 8 cases, and 2 cases of soft tissue tumors Peripheral edge of the bone shell formation, non-urate crystals were seen in 3 cases; 9 cases of cell division as rare, 1 cases of mitotic common and atypia obvious; tumor cell vimentin positive, 5 cases of SMA partially positive, 3 cases of CD34 Some of the tumor cells were positive, the desmin, S100, AE1 / AE3 were negative, Ki67 index (8 cases ≤ 4%, only 1 case 30%); AB / PAS staining: 8 cases of mucinous stromal tumor and perivascular mucosal changes AB staining positive. Conclusion Most of osteomalacia-associated tumors are benign or low-grade mesenchymal tumors. Due to the diversity of histology, they are easily misdiagnosed, and their common features and clinical data can be correctly diagnosed.