Abstract：
Spitz tumors represent a heterogeneous group of challenging melanocytic neoplasms, displaying a range of biological behaviors, spanning from benign lesions, Spitz nevi (SN) to Spitz melanomas (SM), with intermediate lesions in between known as atypical Spitz tumors (AST). They are histologically characterized by large epithelioid and/or spindled melanocytes arranged in fascicles or nests, often associated with characteristic epidermal hyperplasia and fibrovascular stromal changes. In the last decade, the detection of mutually exclusive structural rearrangements involving receptor tyrosine kinases ROS1, ALK, NTRK1, NTRK2, NTRK3, RET, MET, serine threonine kinases BRAF and MAP3K8, or HRAS mutation, led to a clinical, morphological and molecular based classification of Spitz tumors. The recognition of some reproducible histological features can help dermatopathologist in assessing these lesions and can provide clues to predict the underlying molecular driver. In this review, we will focus on clinical and morphological findings in molecular Spitz tumor subgroups.
摘要：
Spitz肿瘤代表一组异质性的具有挑战性的黑素细胞肿瘤，展示了一系列的生物学行为，从良性病变开始，斯皮茨痣（SN）到斯皮茨黑色素瘤（SM），中间病变被称为非典型Spitz肿瘤（AST）。它们的组织学特征是大的上皮样和/或纺锤形黑素细胞排列在束状或巢状，常伴有特征性表皮增生和纤维血管基质改变。在过去的十年里，涉及受体酪氨酸激酶ROS1，ALK，NTRK1,NTRK2,NTRK3,RET,MET,丝氨酸苏氨酸激酶BRAF和MAP3K8或HRAS突变，导致了临床，Spitz肿瘤的形态学和分子分类。识别一些可重复的组织学特征可以帮助皮肤病理学家评估这些病变，并可以提供线索来预测潜在的分子驱动因素。在这次审查中,我们将重点关注Spitz分子肿瘤亚组的临床和形态学发现。
