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斯皮茨瘤的临床、形态学及分子特征

Clinical, Morphological and Molecular Features of Spitz tumors.

作者信息

Donati Michele, Mansour Boulos, Hagstrom Michael, Gerami Pedram, Kazakov Dmitry V

出版信息

Cesk Patol. 2024;60(1):35-48.

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

斯皮茨肿瘤是一组异质性的、具有挑战性的黑素细胞肿瘤,表现出一系列生物学行为,从良性病变斯皮茨痣(SN)到斯皮茨黑色素瘤(SM),其间的中间病变称为非典型斯皮茨肿瘤(AST)。它们在组织学上的特征是大的上皮样和/或梭形黑素细胞排列成束状或巢状,常伴有特征性的表皮增生和纤维血管间质改变。在过去十年中,检测到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的相互排斥的结构重排,或HRAS突变,从而形成了基于临床、形态学和分子的斯皮茨肿瘤分类。认识到一些可重复的组织学特征有助于皮肤病理学家评估这些病变,并可为预测潜在的分子驱动因素提供线索。在本综述中,我们将重点关注分子斯皮茨肿瘤亚组的临床和形态学表现。

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