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致癌性维生素D抵抗性低磷性骨软化症(骨良性骨化性间叶肿瘤):病例报告

Oncogenic vitamin D resistant hypophosphatemic osteomalacia (benign ossifying mesenchymal tumor of bone): case report.

作者信息

Uchida H, Yokoyama S, Kashima K, Nakayama I, Shimizu K, Masumi S

机构信息

First Department of Pathology, Medical College of Oita.

出版信息

Jpn J Clin Oncol. 1991 Jun;21(3):218-26.

PMID:1942553
Abstract

A 53-year-old Japanese woman suffered hypophosphatemic osteomalacia secondary to bone tumor. Her clinical symptoms did not improve for a long time following the oral administration of Alfacalcidol (vitamin D3). A bone survey using radiographs revealed a small tumor located in the proximal head of the left fibula. Hypophosphatemia and low levels of 1-alpha,25-dihydroxycholecalciferol (1-alpha,25(OH)2D3) returned towards normal levels soon after an en-bloc tumor resection, and bone pain and muscle weakness gradually disappeared. The histology, especially of the trabecular bone of the left fibula, showed typical features of osteomalacia with demineralization. The tumor comprised numerous small blood vessels, spindle shaped or oval neoplastic cells, a few multinucleated giant cells, osteoid tissue with or without calcification, chondroid tissue and hemangioma. The spindle shaped or oval cells contained a round nucleus with no mitotic figures. These clinical and histological findings coincide well with a diagnosis of benign ossifying mesenchymal tumor of the bone with vitamin D resistant hypophosphatemic osteomalacia.

摘要

一名53岁的日本女性因骨肿瘤继发低磷性骨软化症。口服阿法骨化醇(维生素D3)后,她的临床症状长期未改善。通过X线片进行的骨骼检查发现左腓骨近端头部有一个小肿瘤。在肿瘤整块切除后不久,低磷血症和1-α,25-二羟胆钙化醇(1-α,25(OH)2D3)水平恢复正常,骨痛和肌肉无力逐渐消失。组织学检查,尤其是左腓骨小梁骨的组织学检查,显示出典型的骨软化伴脱矿特征。肿瘤由大量小血管、梭形或椭圆形肿瘤细胞、少数多核巨细胞、有或无钙化的类骨组织、软骨样组织和血管瘤组成。梭形或椭圆形细胞含有圆形细胞核,无有丝分裂象。这些临床和组织学发现与骨良性骨化性间充质肿瘤伴维生素D抵抗性低磷性骨软化症的诊断非常吻合。

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