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Pediatric-type Myoid Neoplasms of Somatic Soft Tissue: A Clinicopathological and Molecular Genetic Study of 78 Tumors, Highlighting Indolent Clinical Behavior and Frequent SRF Gene Rearrangements.
Mod Pathol
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Department of Pathology, Boston Children's Hospital, Boston, Massachusetts. Electronic address:
Soft tissue tumors with smooth muscle differentiation are rare in pediatric patients. Despite often showing morphologic features sufficient for classification as "leiomyosarcoma" in adults (eg, high cellularity and mitotic activity), clinical follow-up has shown only indolent behavior. The pathological features of recently reported SRF-rearranged "cellular myofibromas/myopericytomas," typically occurring in children, overlap with those of true smooth muscle tumors.
View Article and Find Full Text PDF[SRF-rearranged cellular perivascular myoid tumor: a clinicopathological analysis of two cases].
Zhonghua Bing Li Xue Za Zhi
January 2024
Department of Pathology, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical College, Fudan University; Institute of Pathology, Fudan University, Shanghai 200032, China.
To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed.
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Hum Pathol
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Douglass Hanly Moir Pathology, Macquarie Park, NSW 2113, Australia; School of Medicine, The University of Notre Dame Australia, Darlinghurst, NSW 2010, Australia.
Cellular myofibromas/myopericytomas harboring recurring SRF fusions are recently characterized as rare and diagnostically challenging entities, which can mimic myogenic sarcomas. These tumors belong to the pericytic/perivascular myoid tumor family, which comprises a group of genetically heterogenous and sometimes morphologically overlapping entities. In this series, we describe 3 cases of SRF-rearranged cellular myofibromas/perivascular myoid tumors with a smooth muscle-like phenotype in children.
View Article and Find Full Text PDFExpanding the Spectrum of Perioral Myogenic Tumors in Pediatric Patients: An Fused Perivascular Tumor of the Philtrum.
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Article Synopsis
- Perivascular tumors like myopericytoma and myofibroma are rare benign growths made of smooth muscle cells but can be difficult to diagnose due to resembling more common sarcomas.
- A unique subset of these tumors has been identified with atypical features and specific gene fusions (like SRF::NCOA2), complicating their classification.
- This report describes the first case of such a tumor in a child's lip area, emphasizing its clinical, pathological, and molecular distinctions to prevent misdiagnosis, particularly as spindle cell rhabdomyosarcoma.