Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
The PLEKHH2::ALK fusion is a rarely reported gene fusion identified predominantly in lung adenocarcinomas. Tumors with this fusion have been reported to be of durable response to ALK inhibitors. We herein present the case of a 21-year-old woman with a histomorphologically heterogenous mesenchymal neoplasm of the pelvis, expressing both s100 and CD34, with subsequently identified PLEKHH2::ALK fusion. To our knowledge, only a single mesenchymal neoplasm with this gene fusion has been previously reported. We propose that this tumor represents one with a novel ALK fusion in the emerging family of s100 and CD34 expressing mesenchymal neoplasms with oncogenic kinase alterations akin to NTRK -rearranged mesenchymal neoplasms, rather than inflammatory myofibroblastic tumor. Importantly, this tumor demonstrated a significant response to the ALK inhibitor brigatinib.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1097/PAS.0000000000001887 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Spindle Cell Spindle Sarcoma Harboring a Novel Gene Fusion.
Int J Surg Pathol
September 2025
Department of Pathology, The Thirteenth People's Hospital of Chongqing, Chongqing, China.
Soft tissue sarcomas are a heterogeneous group of malignancies arising from mesenchymal cells. Recent advancements in genomic profiling have identified novel gene fusions in these tumors, offering new insights into their pathogenesis and potential therapeutic targets. Here, we describe a spindle cell sarcoma harboring a novel gene fusion.
View Article and Find Full Text PDFSuperficial Anaplastic Lymphoma Kinase ()-Rearranged Myxoid Spindle Cell Neoplasms: A Case Report and Literature Review.
Int J Surg Pathol
September 2025
Pathology Institute Enge, Zurich, Switzerland.
Anaplastic lymphoma kinase ()-rearranged myxoid spindle cell tumors represent a recently recognized subgroup of kinase fusion-positive mesenchymal neoplasms. These tumors often arise in the superficial soft tissues but may occasionally be found in deeper tissues, or, rarely, within visceral organs. The term (SAMS) comprises a distinct subset of cutaneous soft tissue tumors with superficial location and spindle cell morphology, exhibiting bland cytomorphology and favorable clinical outcomes.
View Article and Find Full Text PDFYAP1::KMT2A-Rearranged Sarcoma: Report of a New Case With Unusual Morphology and Immunohistochemical Features.
Genes Chromosomes Cancer
July 2025
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Recurrent KMT2A and YAP1 related fusions have recently been reported in various mesenchymal neoplasms of different histogenesis. First, YAP1::KMT2A fusions have been described in a subset of MUC4-negative sclerosing epithelioid fibrosarcomas (SEF), while VIM::KMT2A fusions in a handful of cases associated with an undifferentiated spindle cell phenotype lacking stromal hyalinization. On the other hand, YAP1 gene rearrangements have been reported in a wide spectrum of sarcomas, including vascular neoplasms such as epithelioid hemangioendothelioma (EHE).
View Article and Find Full Text PDFCD34-Positive Acral Chondromyxoid Mesenchymal Neoplasm Harboring a Novel TCF4::ERG Fusion.
Genes Chromosomes Cancer
July 2025
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Molecular testing has significantly transformed the field of anatomic pathology over the past several decades. Despite these advances, acral mesenchymal neoplasms remain diagnostically challenging, requiring careful integration of clinical presentation, histologic features, and molecular findings for accurate classification. Herein, we present a case of an acral chondromyxoid mesenchymal neoplasm harboring a novel in-frame TCF4::ERG fusion involving the right index finger of a 26-year-old female.
View Article and Find Full Text PDFUterine Myxoid Mesenchymal Tumor With a Novel SS18::VEZF1 Gene Fusion, Lacking Worrisome Histological Features.
Genes Chromosomes Cancer
July 2025
Molecular Biology Laboratory, Instituto Valenciano de Oncología, Valencia, Spain.
We report a uterine myxoid mesenchymal tumor with a novel SS18::VEZF1 gene fusion. The current lesion was identified in a 53-year-old woman who presented with symptomatic "fibroids" showing accelerated growth and heterogeneous morphology on radiologic assessment. Microscopic examination revealed a well-demarcated neoplasm, and the tumor exhibited alternating hypocellular/hyalinized and hypercellular areas, composed of a monomorphic proliferation of spindle, ovoid, and epithelioid cells arranged in sheets.
View Article and Find Full Text PDF