Locally aggressive and multifocal phosphaturic mesenchymal tumors: two unusual cases of tumor-induced osteomalacia
Locally aggressive and multifocal phosphaturic mesenchymal tumors: two unusual cases of tumor-induced osteomalacia
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DOI：
10.1007/s00256-015-2246-x
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发表时间：
2015-12-01
影响因子：
2.1
通讯作者：
Foss, Erik
中科院分区：
文献类型：
--
作者：
Higley, Meghan;Beckett, Brooke;Foss, Erik
肿瘤引起的骨软化症（TIO）长期以来被认为是一种临床副肿瘤综合征。一个独特的组织病理学实体，磷酸盐尿间充质肿瘤（PMT），作为TIO的独特病因的鉴定是最近的发现。大多数已发表的病例描述了骨软化症患者的孤立、非侵袭性软组织或骨病变;侵袭性或多灶性病变似乎非常罕见。这些肿瘤特征性地分泌成纤维细胞生长因子23（FGF 23）。升高的FGF 23血清水平导致磷酸盐消耗和骨软化。在大多数情况下，实验室异常和临床体征和症状的骨软化症之前确定的致病性病变多年。确诊后，广泛切除以防止局部复发是最常见的治愈方法。PMT的影像学特征多种多样，并且仍然不完全定义，因为大多数既往出版物都在放射学文献之外。我们对两例患有衰弱性骨软化症和异常表现的PMT的患者进行了多种成像检查：其中一例出现局部侵袭性病变导致病理性骨折，第二例出现极其罕见的多灶性PMT。
Tumor-induced osteomalacia (TIO) has long been recognized as a clinical paraneoplastic syndrome. The identification of a unique histopathologic entity, the phosphaturic mesenchymal tumor (PMT), as a distinct etiology for TIO has been a more recent discovery. The majority of published cases describe a solitary, non-aggressive appearing soft tissue or osseous lesions in patients with osteomalacia; aggressive appearing or multifocal lesions appear to be exceedingly rare. These tumors characteristically secrete fibroblast growth factor 23 (FGF23). Elevated serum levels of FGF23 result in phosphate wasting and osteomalacia. In the majority of cases, laboratory abnormalities and clinical signs and symptoms of osteomalacia precede identification of the causative lesion by years. Following diagnosis, complete resection with wide margins to prevent local recurrence is most often curative. Imaging characteristics of PMT are diverse and remain incompletely defined, as the majority of previous publications are outside of the radiologic literature. We present multiple imaging modalities in two cases of patients with debilitating osteomalacia and unusual appearing PMTs: one with a locally aggressive lesion leading to pathologic fracture, the second presenting with exceedingly rare multifocal PMT.