[Clinicopathologic study of 10 cases of osteomalacia or rickets-associated mesenchymal tumors].
[Clinicopathologic study of 10 cases of osteomalacia or rickets-associated mesenchymal tumors].
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DOI：
10.3760/j.issn:0529-5807.2005.11.006
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发表时间：
2005-11
期刊：
影响因子：
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通讯作者：
D. Zhong;Tong-hua Liu;Di Yang;R. Feng;Q. Cui;Yufeng Luo;Yong Jia
中科院分区：
文献类型：
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作者：
D. Zhong;Tong-hua Liu;Di Yang;R. Feng;Q. Cui;Yufeng Luo;Yong Jia
目的探讨骨软化症或佝偻病相关间充质肿瘤的临床病理特征。方法对10例骨软化或佝偻病相关间充质肿瘤的临床和病理表现进行分析。对档案石蜡切片进行苏木精、伊红染色、免疫组织化学和组织化学染色。结果10例患者中，男性6例，女性4例。手术时年龄28 ~ 69岁，平均45.6岁。所有病例均有长期骨痛、关节痛、活动受限、低磷血症和高磷尿病史。症状持续时间为2 ~ 27年（平均9.6年）。肿瘤大小1 ~ 7cm，平均3.52 cm。显微镜下，肿瘤由多种间充质细胞组成，包括纺锤状成纤维细胞样细胞、脂肪细胞、软骨样细胞和粘液细胞。背景是丰富的血管。10例中有8例伴有营养不良性钙化，呈不寻常的絮状或“脏污”状。2例外周编织骨壳形成，2例非尿酸结晶沉积。有丝分裂象少见9例。10例中有1例常见有丝分裂象和奇异细胞。免疫组化结果显示，肿瘤细胞均呈波形蛋白阳性。平滑肌肌动蛋白局灶阳性5例，CD34局灶阳性3例。desmin、S-100、AE1/AE3染色均为阴性。Ki-67增殖指数低于4% 8例，30% 1例。阿利新蓝阳性黏液基质及血管周围黏液变性8例。结论骨软化症或佝偻病相关肿瘤多数为良性或低度恶性间质肿瘤。由于形态学的异质性，它们可能被误认为是其他肿瘤。彻底了解相关的临床特征和实验室检查结果有助于得出正确的诊断。
OBJECTIVE To study the clinicopathologic features of osteomalacia or rickets-associated mesenchymal tumors. METHODS The clinical and pathologic findings of 10 cases of osteomalacia or rickets-associated mesenchymal tumors were evaluated. Hematoxylin and eosin stain, 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 28 to 69 years ( mean = 45.6 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 2 to 27 years (mean = 9.6 years). The tumor size ranged from 1 to 7 cm (mean size = 3.52 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 8 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 and non-urate crystal deposition in 2 cases. Mitotic figures were rare in 9 cases. In 1 of the 10 cases however, mitotic figures and bizarre cells were commonly encountered. On immunohistochemical study, the tumor cells were all positive for vimentin. There was focal positivity for smooth muscle actin and CD34 in 5 and 3 cases respectively. The staining for desmin, S-100 and AE1/AE3 was negative. Ki-67 proliferation index was less than 4% in 8 cases and 30% in 1 case. Alcian blue-positive mucinous matrix and mucinous degeneration around vessels were noted in 8 cases. CONCLUSIONS Most of the osteomalacia or rickets-associated 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.