Angeles-Angeles Arturo, Reza-Albarrán Alfredo, Chable-Montero Fredy, Cordova-Ramón Juan Carlos, Albores-Saavedra Jorge, Martinez-Benitez Braulio
Department of Pathology, Instituto Nacional de Ciencias Médicas y la Nutrición "Salvador Zubirán", Mexico City, Mexico.
Department of Internal Medicine, Instituto Nacional de Ciencias Médicas y la Nutrición "Salvador Zubirán", Mexico City, Mexico.
Ann Diagn Pathol. 2015 Dec;19(6):375-80. doi: 10.1016/j.anndiagpath.2015.08.003. Epub 2015 Aug 28.
Phosphaturic mesenchymal tumor (PMT) is a morphologically heterogeneous soft tissue and bone neoplasm, producing a paraneoplastic syndrome due to phosphate wasting. These tumors produce fibroblast growth factor 23, which is implicated in renal tubule phosphate loss. Medical records of patients seen from 1999 to 2013 with osteomalacia associated or not with a tumor were reviewed. Clinical and laboratory data, radiographic studies, and follow-up of 8 patients were tabulated. Histologic features and the immunoprofile of the tumors were analyzed. There were 208 patients with osteomalacia, but only 8 (3.84%) had osteomalacia associated with a tumor. The median age of the patients was 40 years. The tumor size ranged from 1.5 to 4 cm. Five were located in soft tissues and skin; and 3, in bones. Osteomalacia symptoms lasted from 2 to 14 years with a median of 6 years. Laboratory data showed hypophosphatemia and phosphaturia in all patients. All tumors were histologically benign. Histologically, the salient features were a hemangiopericytoid pattern, chronic hemorrhage, and microcystic areas. All neoplasms were diffusely positive for vimentin and focally positive for epithelial membrane antigen, CD34, and S-100 protein. Ki-67 was positive in approximately 10% of neoplastic cells in 2 cases and less than 1% in the remainder. We report 8 cases of PMTs producing osteomalacia, from a single third-level Mexican medical institution. These tumors occurred in soft tissues, skin, and bones. All tumors were benign, small, not easily detected by physical examination and diagnosed due to the metabolic abnormalities.
促排磷性间叶肿瘤(PMT)是一种形态学上异质性的软组织和骨肿瘤,因磷酸盐消耗而产生副肿瘤综合征。这些肿瘤产生成纤维细胞生长因子23,这与肾小管磷酸盐丢失有关。回顾了1999年至2013年期间就诊的伴有或不伴有肿瘤的骨软化症患者的病历。将8例患者的临床和实验室数据、影像学研究及随访情况制成表格。分析了肿瘤的组织学特征和免疫表型。有208例骨软化症患者,但只有8例(3.84%)的骨软化症与肿瘤相关。患者的中位年龄为40岁。肿瘤大小在1.5至4厘米之间。5例位于软组织和皮肤;3例位于骨骼。骨软化症症状持续2至14年,中位时间为6年。实验室数据显示所有患者均有低磷血症和磷酸盐尿。所有肿瘤在组织学上均为良性。组织学上,主要特征为血管外皮细胞瘤样模式、慢性出血和微囊区。所有肿瘤波形蛋白弥漫性阳性,上皮膜抗原、CD34和S-100蛋白局灶性阳性。2例中约10%的肿瘤细胞Ki-67呈阳性,其余病例中该比例小于1%。我们报告了来自墨西哥一家三级医疗机构的8例导致骨软化症的PMT病例。这些肿瘤发生于软组织、皮肤和骨骼。所有肿瘤均为良性,体积小,体格检查不易发现,因代谢异常而得以诊断。