上颌骨混合性上皮–结缔组织型磷酸盐尿性间叶性肿瘤1例

A Case of Mixed Epithelial-Connective Tissue Type Phosphaturic Mesenchymal Tumor of the Maxilla

摘要: 目的：报道1例上颌骨混合性上皮–结缔组织型磷酸盐尿性间叶性肿瘤(PMTMECT)患者的临床病理特征、治疗及预后。方法：回顾性分析1例PMTMECT患者的临床表现、病理特征、治疗及预后，并进行文献复习。结果：31岁男性患者，双侧髋关节疼痛加重伴腰背部疼痛入院。组织学形态结果显示：肿瘤组织由上皮和间叶两种成分组成，间叶成分由梭形细胞构成，肿瘤细胞呈血管外皮瘤样排列，可见“污浊”或絮状钙化基质。上皮成分类似牙源性上皮，以片状或不规则巢状广泛分布在肿瘤间。免疫组化染色显示肿瘤细胞表达间叶标志物Vimentin、上皮标志物AE1/AE3、Ki-67指数约5%。病理诊断为PMTMECT后，患者经手术切除治疗，目前随访2年，病情未复发。结论：PMTMECT肿瘤体积一般较小，临床无特异性表现，易漏诊，手术切除效果好。

Abstract: Objective: To report the clinicopathological features, treatment and prognosis of a patient with mixed epithelial-connective tissue-type phosphaturic mesenchymal tumor of the maxilla (PMTMECT). Methods: The clinical presentation, pathological features, treatment and prognosis of a patient with PMTMECT were retrospectively analyzed, and the literature was reviewed. Results: A 31-year-old male patient with increased bilateral hip pain with low back pain was admitted to the hospital. The histological findings showed that the tumor tissue consisted of both epithelial and mesenchymal components, with the mesenchymal component consisting of spindle-shaped cells, and the tumor cells were arranged in a hemangiopericytoma-like pattern, with a “stained” or floc-culent calcified stroma. The epithelial component resembled odontogenic epithelium and was widely distributed among the tumors in sheets or irregular nests. Immunohistochemical staining showed that the index of tumor cells expressed mesenchymal marker Vimentin, epithelial marker AE1/AE3, and Ki-67 is about 5%. After pathological diagnosis of PMTMECT, the patient was treated with surgical resection and is currently followed up for 2 years with no recurrence of disease. Con-clusion: PMTMECT tumors are generally small in size, have no specific clinical manifestations, are easily missed, and have good surgical resection results.