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When Benign Tumors Mimic Malignancies: A Case of Lymphangiomatosis Masquerading as Metastatic Disease

机译:当良性肿瘤模拟恶性肿瘤:淋巴瘤病假装为转移性疾病的一例。

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Lymphangiomatosis, a rare disorder of the lymphatic system characterized by the abnormal proliferation of lymphatic vessels, is a typically benign disorder that at times can exhibit invasive or malignant behavior. While generally considered a diagnosis of childhood, in adults the majority of cases are asymptomatic and found incidentally. Rarely, lymphatic overgrowth can occur, causing growth of lesions on imaging mimicking a metastatic process and occasionally, resulting in substantial morbidity and mortality. Here, we present such a case of lymphangiomatosis with multi-organ system involvement in liver, bone, and spleen. In addition to details of the clinical presentation and the pathologic review which led to the diagnosis, we describe our use of the tyrosine kinase inhibitor pazopanib, which may cause stabilization of lymphangiomatosis through blockade of vascular endothelial growth factor (VEGF) signaling, for systemic treatment in this unusual case.
机译:淋巴管瘤病是一种以淋巴管异常增生为特征的罕见淋巴系统疾病,是典型的良性疾病,有时会表现出侵袭性或恶性行为。虽然通常被认为可以诊断儿童期,但在成年人中,大多数病例是无症状的,并且是偶然发现的。很少会发生淋巴过度生长,从而在模仿转移过程的影像上引起病变的发展,并偶尔导致相当大的发病率和死亡率。在这里,我们介绍了这样一种淋巴管瘤病,多器官系统累及肝,骨和脾脏。除导致诊断的临床表现和病理学检查的详细信息外,我们还描述了酪氨酸激酶抑制剂帕唑帕尼的使用,该药物可通过阻断血管内皮生长因子(VEGF)信号来稳定淋巴管瘤病,用于全身治疗在这种不寻常的情况下。

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