Diagnostic and Interventional Radiology Unit with Integrated Section of Translational Medical Oncology, National Cancer Research Centre Istituto Tumori "Giovanni Paolo II", Bari, Italy.
Diagnostic and Interventional Radiology Unit with Integrated Section of Translational Medical Oncology, National Cancer Research Centre Istituto Tumori "Giovanni Paolo II", Bari, Italy.
Crit Rev Oncol Hematol. 2015 Mar;93(3):159-69. doi: 10.1016/j.critrevonc.2014.10.010. Epub 2014 Oct 31.
Human mastocytosis are heterogeneous group of neoplastic diseases characterized by a different degree of uncontrolled mast cell (MC) proliferation and activation. Interestingly, human mastocytosis share several biological and clinical features with canine mast cell disorders, so called canine mast cell tumors (CMCTs). These CMCTs are the most common spontaneous cutaneous tumors found in dogs representing a valid model to study neoplastic mast cell disorders. It has been discovered that the pathological activation of c-Kit receptor (c-KitR), expressed by MCs, has been involved in the pathogenesis of neoplastic MC disorders. In this review we have focused on human mastocytosis in terms of: (i) epidemiology and classification; (ii) pathogenesis at molecular levels; (iii) clinical presentation. In addition, we have summarized animal models useful to study neoplastic MC disorders including CMCTs and murine transgenic models. Finally, we have revised therapeutic approaches mostly common in human and canine MCTs and novel tyrosine kinase inhibitors approved for CMCTs and recently translated in human clinical trials.
人类肥大细胞增多症是一组异质性的肿瘤性疾病，其特征为不同程度的不可控肥大细胞（MC）增殖和激活。有趣的是，人类肥大细胞增多症与犬肥大细胞瘤（CMCT）具有一些共同的生物学和临床特征。这些 CMCT 是犬中最常见的自发性皮肤肿瘤，是研究肥大细胞瘤性疾病的有效模型。已经发现，表达于 MC 的 c-Kit 受体（c-KitR）的病理性激活与肿瘤性 MC 疾病的发病机制有关。在这篇综述中，我们重点关注了人类肥大细胞增多症，包括：（i）流行病学和分类；（ii）分子水平上的发病机制；（iii）临床表现。此外，我们还总结了用于研究肥大细胞瘤性疾病的动物模型，包括 CMCT 和鼠转基因模型。最后，我们复习了人类和犬肥大细胞瘤中常用的治疗方法，以及最近在 CMCT 中获得批准并转化为人类临床试验的新型酪氨酸激酶抑制剂。
