Clinical, morphologic, and genomic findings in ROS1 fusion Spitz neoplasms Journal Article


Authors: Gerami, P.; Kim, D.; Compres, E. V.; Zhang, B.; Khan, A. U.; Sunshine, J. C.; Quan, V. L.; Busam, K.
Article Title: Clinical, morphologic, and genomic findings in ROS1 fusion Spitz neoplasms
Abstract: The presence of a characteristic chimeric fusion as the initiating genomic event is one defining feature of Spitz neoplasms. Characterization of specific subtypes of Spitz neoplasms allows for better recognition facilitating diagnosis. Data on clinical outcomes of the specific tumor types may help in predicting behavior. In this study we present the largest series to date on ROS1 fusion Spitz neoplasms. We present the clinical, morphologic, and genomic features of 17 cases. We compared the morphologic features of these 17 cases to a cohort of 99 other non-ROS1 Spitz neoplasms to assess for features that may have high specificity for ROS1 fusions. These tumors consisted of ten Spitz nevi and seven Spitz tumors. None of the cases met criteria for a diagnosis of Spitz melanoma. Morphologically, the ROS1 fusion tumors of this series were characterized by a plaque-like or nodular silhouette, often densely cellular intraepidermal melanocyte proliferation, frequent pagetosis, tendency toward spindle cell cytomorphology, low grade nuclear atypia, and floating nests with occasional transepidermal elimination. However, there was a significant range in microscopic appearances, including two cases with morphologic features of a desmoplastic Spitz nevus. Different binding partners to ROS1 were identified with PWWP2A and TPM3 being the most common. No case had a recurrence or metastasis. Our findings document that most ROS1 fusion Spitz neoplasms have some typical characteristic microscopic features, while a small proportion will have features overlapping with other genomic subtypes of Spitz neoplasms. Preliminary evidence suggests that they tend to be indolent or low grade neoplasms. © 2020, The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.
Journal Title: Modern Pathology
Volume: 34
Issue: 2
ISSN: 0893-3952
Publisher: Nature Research  
Date Published: 2021-02-01
Start Page: 348
End Page: 357
Language: English
DOI: 10.1038/s41379-020-00658-w
PUBMED: 32862201
PROVIDER: scopus
PMCID: PMC7855005
DOI/URL:
Notes: Article -- Export Date: 1 February 2021 -- Source: Scopus
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  1. Klaus J Busam
    689 Busam