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致癌性骨软化症:17例骨病变的临床病理研究

Oncogenic osteomalacia: a clinicopathologic study of 17 bone lesions.

作者信息

Park Y K, Unni K K, Beabout J W, Hodgson S F

机构信息

Section of Surgical Pathology, Kyung Hee University Medical Center, Korea.

出版信息

J Korean Med Sci. 1994 Aug;9(4):289-98. doi: 10.3346/jkms.1994.9.4.289.

DOI:10.3346/jkms.1994.9.4.289
PMID:7848576
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3054097/
Abstract

Oncogenic osteomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase. We collected from the Mayo Clinic files and from our consultation files the records for 17 cases of osteomalacia associated with bone lesions. There were five cases of fibrous dysplasia, three of hemangiopericytoma, and two of phosphaturic mesenchymal tumor. There was one case each of osteosarcoma, chondroblastoma, chondromyxoid fibroma, malignant fibrous histiocytoma, giant cell tumor, metaphyseal fibrous defect, and hemangioma. In this study we can figure out that the most common characteristic histologic features of our cases were hemangiopericytomatous vascular proliferation, fine lace-like stromal calcification, and stromal giant cells. In most of the cases, the clinical and biochemical symptoms and signs resolved soon after complete resection of the lesion. When the lesion recurred or metastasized, the symptoms and signs also recurred.

摘要

致癌性骨软化症是一种罕见的临床病理综合征,其特征为间充质肿瘤,该肿瘤显然会导致骨软化症以及由低磷血症、血钙正常和碱性磷酸酶水平升高组成的生化异常。我们从梅奥诊所档案和会诊档案中收集了17例与骨病变相关的骨软化症病例记录。其中有5例纤维发育不良、3例血管外皮细胞瘤和2例排磷性间充质肿瘤。骨肉瘤、软骨母细胞瘤、软骨黏液样纤维瘤恶性纤维组织细胞瘤、巨细胞瘤、干骺端纤维性缺损和血管瘤各有1例。在本研究中,我们可以发现,我们病例最常见的特征性组织学表现为血管外皮细胞瘤样血管增生、细花边样基质钙化和基质巨细胞。在大多数病例中,病变完全切除后,临床和生化症状及体征很快消失。当病变复发或转移时,症状和体征也会复发。

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