melanocytomas

  • DOI:
    文章类型: Journal Article
    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。
    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.
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  • DOI:
    文章类型: Journal Article
    黑素细胞性病变是不稳定的肿瘤,其基因组及其变化决定了它们的形态和生物学特性。中间病变具有两者的组织形态学特征,痣和黑色素瘤。黑色素细胞瘤代表根据最近的分子生物学研究分离的一组。这篇文章总结了良性的,中间,恶性和合并黑素细胞皮肤病变,并提供实用的诊断建议。
    Melanocytic lesions are instable tumors, the genome of which and its changes determinate their morphology and biological properties. Intermediate lesions share histomorphological features of both, nevi and melanoma. Melanocytomas represent a group of them separated on the basis of recent molecular-biological studies. The article summarizes benign, intermediate, malignant and combined melanocytic skin lesions and offers practical recommendations for diagnosis.
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  • 文章类型: Journal Article
    Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. The aim of this study was to investigate the clinical manifestations, radiological features, management, and follow-up data of spinal meningeal melanocytomas. We present the clinical data and long-term outcomes from a consecutive surgical series of 16 patients with pathologically diagnosed spinal meningeal melanocytomas. All of the patients underwent surgical resection. Pre and postoperative MRI was performed. Follow-up data and neurological functional assessment is presented and discussed. The mean age at diagnosis was 42.0 years, with a significant male predominance. The primary clinical symptoms were weakness or numbness of the extremities. The appearance of melanocytoma on MRI is typically isointense to hyperintense on T1-weighted images, hypointense on T2-weighted images, and contrast enhancement tends to be remarkable and homogeneous. In most cases, gross total resection is achievable; however, in rare cases with dumbbell-shaped tumors involving the extraspinal region, a staging operation and subtotal resection should be attempted. During a mean follow-up period of 58.1 months, the symptoms were completely relieved in all the patients, and no tumor progression or recurrence was noted. Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.
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