摘要
目的研究磷酸盐尿性间叶肿瘤(混合结缔组织亚型)的临床与病理学特点。方法对1例骨软化症患者的肿块切除标本进行光镜、电镜和免疫组化SP法检测。结合临床资料,并复习相关文献。结果患者表现为顽固性骨软化症、低磷酸盐血症,高磷酸盐尿,高血清碱性磷酸酶,外周血单核细胞增多,血钙浓度正常。经99mTc-OCT(奥曲肽)检查指导临床发现左腘窝区小肿块并切除之。术后3天,血磷恢复正常。光镜下肿瘤主要由肥胖的梭形细胞和破骨细胞样多核巨细胞构成,具有丰富的血管,可见血管外皮瘤样血管、散在脂肪岛、明显的出血及含铁血黄素沉积,肿瘤边缘有不完整的膜状骨样或软骨样组织。电镜下见梭形细胞内含数量不等的细颗粒状电子致密物,其中部分为结晶样高电子致密度颗粒。免疫组化显示多核细胞和单核间质细胞CD68阳性。结论该例为肿瘤源性骨软化症,其病理类型为磷酸盐尿性间叶肿瘤,混合结缔组织亚型。外科切除后治愈,99mTc-OCT对于指导临床发现小的隐蔽病灶很有价值。
Purpose To study the clinical and pathological features of the phosphaturic mesenchymal tumor, mixed connective tissue variant（PMT/MCT）. Methods The tumor resected from a patient with osteomalacia was studied by light and electron microscopy and immunohistochemistry, and the related literature was reviewed as well Results The clinical feature was intractable osteomalacia characterized by hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase, monocytosis, and normal serum concentrations of calcium. 99mTc-OCT examination suggested that the tumor was located in the left popliteal fossa, and three days after remove of the tumor, the serum phosphate level returned to normal. Microscopically, the tumor was primarily composed of numerous plump spindle cells and osteoclast-like giant cells, and was rich in vasculature. In addition, there were scattered islands of mature adipocytes, hemangiopericytoma-like vessels, osteoid and calcified cartilage-like tissue, hemorrhage and obvious hemosiderin deposition were observed as well. The tumor had no border, and an incomplete rim of membranous osteoid or cartilage-like tissue could be seen. Under electron microscope, micro-granular electron-dense matter was seen in the spindle cells, some were crystal-like, which were either free or combined with lysosome. No neuroendocrine granule was found. The tumor cells expressed CD68, α-SMA and vimentin, whereas they were negative for all other markers. Conclusions The case reported here is an oncogenic osteomalacia, and its histopathologic type is PMT/MCT. Surgical resection can effectively cure the disease, and 99mTc-OCT is valuable to show the location of the tumor even if the tumor is small and occult.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2007年第5期557-561,共5页
Chinese Journal of Clinical and Experimental Pathology
