CAJ | 학술논문

目的：探讨磷酸盐尿性间叶肿瘤的临床病理学特点。方法回顾分析10例患者的临床资料，观察10例磷酸盐尿性间叶肿瘤的形态及免疫表型。结果患者男6例，女4例，年龄范围23～55岁，平均43.1岁；患者均有1～13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0.8～5 cm不等（平均3.05 cm）；瘤组织为间叶组织来源，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，4例病变中有少见的絮状或不规则砂砾样钙盐沉积，3例发生于软组织的肿瘤周边见骨壳形成；8例细胞分裂象少见，2例核分裂象多见并且异型性明显；瘤细胞VIM及CD56阳性，7例NSE阳性，3例Bcl-2瘤细胞阳性，结蛋白、S-100、AE1/AE3均阴性，Ki-67指数8例1%～5%，仅2例为20%～30%。结论磷酸盐尿性间叶肿瘤多为良性或低度恶性的间叶组织肿瘤，因组织学多种多样而易误诊，掌握其共同的特征性并结合临床资料方能正确诊断。
목적：탐토린산염뇨성간협종류적림상병이학특점。방법회고분석10례환자적림상자료，관찰10례린산염뇨성간협종류적형태급면역표형。결과환자남6례，녀4례，년령범위23～55세，평균43.1세；환자균유1～13년골통、관절통화활동곤난적병사，검사발현저혈린、고뇨린；종류최대경0.8～5 cm불등（평균3.05 cm）；류조직위간협조직래원，가견다소불등적사형섬유모세포양세포、지방세포、연골양세포、점액양세포등，류조직부우혈관，4례병변중유소견적서상혹불규칙사력양개염침적，3례발생우연조직적종류주변견골각형성；8례세포분렬상소견，2례핵분렬상다견병차이형성명현；류세포VIM급CD56양성，7례NSE양성，3례Bcl-2류세포양성，결단백、S-100、AE1/AE3균음성，Ki-67지수8례1%～5%，부2례위20%～30%。결론린산염뇨성간협종류다위량성혹저도악성적간협조직종류，인조직학다충다양이역오진，장악기공동적특정성병결합림상자료방능정학진단。
Objective To study the clinicopathologic features of phosphaturic mesenchymal tumor. Methods The clinical and pathologic findings of 10 cases of phosphaturic mesenchymal tumor were evaluated. Hematoxylin and eosinstain, immunohistochemistry and histochemistry were performed on the archival paraffin sections. Results Amongst the 10 patients studied, 6 were males and 4 were females. Their age at the time of operation ranged from 23 to 55 years (mean=43.1 years). A history of long standing bone pain, arthralgia, limitation in movement, hypophosphatemia and hyperphosphaturia was present in all cases. The duration of symptoms ranged from 1 to 13 years. The tumor size ranged from 0.8 to 5 cm (mean size=3.05 cm). Microscopically, the tumors were composed of various mesenchymal cells, including spindled fibroblast-like cells, adipocytes, chondroid cells and mucinous cells. The background was rich in blood vessels. In 4 of the 10 cases, there was also dystrophic calcification in an unusual flocculent or“grungy”pattern. Peripheral woven bone shell formation was noted in 2 cases. Mitotic figures were rare in 5 cases. In 2 of the 10 cases however, mitotic figures and bizarre cells were commonly encountered. On immunohistochemical study, the tumor cells were all positive for vimentin and CD56. There was focal positivity for NSE and Bcl-2 in 7 and 3 cases respectively. The staining for desmin, S-100 and AE1/A E3 was negative. Ki-67 proliferation index was 1%-5%in 8 cases and 20%-30%in 2 cases. Conclusions Most of the phosphaturic mesenchymal tumors are either benign or low grade malignant mesenchymal tumors. They can be mistaken as other neoplasms due to the morphologic heterogeneity present. Thorough understanding of the associated clinical features and laboratory investigation results is helpful in arriving at the correct diagnosis.