磷酸间充质肿瘤(PMT)是一种罕见的肿瘤,可以通过过度产生FGF23来引起肿瘤诱导的骨软化症(TIO),FGF23是一种导致肾磷酸盐消耗和成骨细胞活性降低的肽激素。由于呈现的症状是非特异性的,因此难以进行PMT的诊断。虽然PMT是一个罕见的实体,大多数病例本质上是良性的,手术后不需要进一步干预,因为切除通常是治愈性的。这里,我们介绍了一例独特的恶性PMT伴从头肝转移的女性患者,该患者出现TIO,并接受了原发病灶的手术切除,随后肝转移消退.此外,我们分析了文献综述,并讨论了及时诊断这种罕见现象的重要性。值得鼓励的是,提供者强烈考虑在其他原因不明的骨痛患者中诊断为PMT,疲劳,弱点,特别是如果伴有低磷酸盐血症。还需要进一步的研究来确定预测PMT恶性潜能的预后因素,因为它们可能有助于确定可能的治疗靶点。
磷酸间充质肿瘤扩散至肝脏:1例报告和文献综述磷酸间充质肿瘤(PMTs)是一种罕见的肿瘤,可通过FGF23信号的过量产生而导致骨丢失。这种激素会导致肾脏失去磷酸盐,并降低身体建立新骨的能力。PMT非常罕见且难以诊断,特别是因为可以在几种不同的疾病中看到呈现的症状。虽然PMT很少见,大多数病例是良性的,只需要做手术.我们正在介绍一个独特的病例,一个患者在她的右大腿出现PMT,并有证据表明疾病传播到她的肝脏。我们的患者接受了大腿病变的手术切除,随后肝脏病变得到了改善。在我们的研究中,我们分析了文献综述,并讨论了及时诊断这种罕见现象的重要性。值得鼓励的是,提供者强烈考虑在其他原因不明的骨痛患者中诊断为PMT,疲劳,弱点,特别是如果伴有低磷酸盐水平。还需要进一步的研究来确定预测PMT恶性潜力的预后因素,因为它们可能有助于确定可能的治疗靶标。
Phosphaturic mesenchymal tumors (PMTs) are rare tumors that can cause tumor-induced osteomalacia (TIO) through overproduction of FGF23, a peptide hormone that causes renal phosphate wasting and reduced osteoblastic activity. The diagnosis of PMTs can be difficult to make as the presenting symptoms are non-specific. Although PMT is a rare entity, most cases are benign in nature, not requiring further intervention after surgery, as resection is typically curative. Here, we present a unique case of malignant PMT with de novo liver metastasis in a female patient who presented with TIO and underwent surgical resection of her primary lesion with subsequent regression of her liver metastasis. Moreover, we analyze a
review of literature and discuss the importance of a timely diagnosis of this rare phenomenon. It is encouraged that providers strongly consider a diagnosis of PMT in patients with otherwise unexplained bone pain, fatigue, weakness, especially if accompanied with hypophosphatemia. Further studies are also warranted to identify prognostic factors that predict a PMT\'s malignant potential as they may help identify possible therapeutic targets.
Phosphaturic mesenchymal tumor with spread to the liver: A case report and literature
review Phosphaturic mesenchymal tumors (PMTs) are rare tumors that can cause bone loss through the excess production of a signal named FGF23. This hormone causes the kidneys to lose phosphate and reduces the body’s ability to build new bone. PMTs are incredibly rare and difficult to diagnose especially since the presenting symptoms can be seen in several different diseases. Although PMT is rare, most cases are benign, only requiring surgery. We are presenting a unique case of a patient who presented with PMT in her right thigh and had evidence that the disease spread to her liver. Our patient underwent surgical resection of her thigh lesion and subsequently had improvement of her liver lesion. In our study, we analyze a
review of literature and discuss the importance of a timely diagnosis of this rare phenomenon. It is encouraged that providers strongly consider a diagnosis of PMT in patients with otherwise unexplained bone pain, fatigue, weakness, especially if accompanied by low phosphate levels. Further studies are also warranted to identify prognostic factors that predict a PMT’s malignant potential as they may help identify possible therapeutic targets.