Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck
Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck
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DOI：
10.1007/s12105-015-0668-3
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发表时间：
2016-06
影响因子：
2.1
通讯作者：
H. Qari;Aya Hamao-Sakamoto;Clay Fuselier;Y. Cheng;H. Kessler;John M. Wright
中科院分区：
文献类型：
--
作者：
H. Qari;Aya Hamao-Sakamoto;Clay Fuselier;Y. Cheng;H. Kessler;John M. Wright
磷酸间充质瘤(PMT)是一种分泌成纤维细胞生长因子-23(成纤维细胞生长因子-23)并导致致癌性骨软化的罕见肿瘤。它发生在成年人中，性别分布均等，最常见的位置是下肢，其次是头部和颈部。除了骨软化外，临床表现还包括骨痛和多发性骨折。显微特征包括梭形细胞、多核巨细胞和嵌入软骨粘液基质的钙化。实验室结果显示正常的钙和甲状旁腺水平，低磷血症，以及升高的成纤维细胞生长因子-23水平，通常在手术切除后恢复正常。由于其罕见，本研究的目的是报告2例新的口腔PMT病例，并回顾头颈部的文献。第一例发生在牙龈，已经存在6年了。第二个病例是先前诊断的右侧下颌骨PMT复发，转移到肺和软组织。文献复习包括头颈部53例。与口内部位(24%)相比，口外部位(76%)更受偏爱，副鼻窦被认为是最常见的部位(38%)，其次是下颌(15%)。9例复发，其中3例恶性，提示为潜在侵袭性肿瘤。由于PMT的生物学行为不确定，且其罕见，对医学、实验室、放射学和组织学结果的综合评估对于明确诊断和治疗至关重要。
Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that secretes fibroblast growth factor-23 (FGF-23) and causes oncogenic osteomalacia. It occurs in adults with equal gender distribution and the most common location is the lower extremities, followed by the head and neck. Besides osteomalacia, the clinical presentation includes bone pain and multiple bone fractures. Microscopic features consist of spindle cells, multinucleated giant cells, and calcifications embedded in a chondromyxoid matrix. Laboratory findings indicate normal calcium and parathyroid levels, hypophosphatemia, and increased levels of FGF-23 that usually revert to normal after surgical removal. Due to its rarity, the purpose of the study was to report 2 new oral cases of PMT and to review the literature in the head and neck. The first case occurred in the gingiva and had been present for 6 years. The second case was a recurrence of a previously diagnosed PMT in the right mandible that metastasized to the lung and soft tissue. The literature review included 53 cases in the head and neck. There was a predilection for extra-oral sites (76 %) compared to intra-oral sites (24 %) with paranasal sinuses considered the most common location (38 %) followed by the mandible (15 %). There were 9 recurrences that included 3 malignant cases indicating a potentially aggressive tumor. Due to the indeterminate biological behavior of PMT and its rarity, a comprehensive evaluation of medical, laboratory, radiographic, and histological findings are crucial for a definitive diagnosis and treatment.