ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2018
INSM1 expression and its diagnostic significance in extraskeletal myxoid chondrosarcoma.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 78 citations in OpenAlex.
- Immunoreactivity for CD34, Desmin, Keratins, KIT, Alpha-Smooth Muscle Actin, S100, and Vimentin in Malignant Mesenchymal Neoplasms in Guinea Pigs: A Series of 62 Cases From a Single Institution.Veterinary medicine and science · 2026Article
- A comprehensive review of ossifying fibromyxoid tumor: insights into its clinical, pathological, and molecular landscape.Journal of pathology and translational medicine · 2026Article
- Novel HSPA8-NR4A2 rearrangement in extraskeletal myxoid chondrosarcoma: a sarcoma mimicker of neuroendocrine neoplasia.Virchows Archiv : an international journal of pathology · 2025Article
- ZC3H7A/B::BCOR fusion fibromyxoid sarcoma of soft tissue: an emerging aggressive sarcoma overlapping with malignant ossifying fibromyxoid tumors.Virchows Archiv : an international journal of pathology · 2025Article
- From pathogenesis to the patient's bedside: a comprehensive review of extraskeletal myxoid chondrosarcoma.Journal of cancer research and clinical oncology · 2025Review
- Expression of insulinoma-associated protein 1 (INSM1) in gastric neuroendocrine and non-neuroendocrine neoplasms.Discover oncology · 2025Article
- Framework for the Pathology Workup of Metastatic Castration-Resistant Prostate Cancer Biopsies.Clinical cancer research : an official journal of the American Association for Cancer Research · 2025Review
- PITX1 as a grading, prognostic and tumor-infiltrating immune cells marker for chondrosarcoma: a public database-based immunoassay and tissue sample analysis.Frontiers in oncology · 2025Article
- A potential conundrum in dermatopathology: molecularly confirmed superficial ossifying fibromyxoid tumors with unusual histomorphologic findings and a novel fusion.Virchows Archiv : an international journal of pathology · 2024Article
- Sarcomas with EWSR1::Non-ETS Fusion (EWSR1::NFATC2 and EWSR1::PATZ1).Surgical pathology clinics · 2024Review
- Extraskeletal myxoid chondrosarcoma: p53 and Ki-67 offer prognostic value for clinical outcome - an immunohistochemical and molecular analysis of 31 cases.Virchows Archiv : an international journal of pathology · 2023Article
- The Complex Histopathological and Immunohistochemical Spectrum of Neuroendocrine Tumors-An Overview of the Latest Classifications.International journal of molecular sciences · 2023Review
- Extraskeletal myxoid chondrosarcoma of the buttock: a case report and literature review.Frontiers in oncology · 2023Article
- Primary intracranial extraskeletal myxoid chondrosarcoma: A case report and review of literature.World journal of clinical cases · 2022Article
- Mutation of KIT in cellular extraskeletal myxoid chondrosarcoma: a case report and literature review.Diagnostic pathology · 2022Review
- Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Soft Tissue Tumors.Head and neck pathology · 2022Review
- INSM1 Expression in Mesenchymal Tumors and Its Clinicopathological Significance.BioMed research international · 2022Article
- Lung neuroendocrine neoplasms: recent progress and persistent challenges.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Review
- INSM1, a Novel Biomarker for Detection of Neuroendocrine Neoplasms: Cytopathologists' View.Diagnostics (Basel, Switzerland) · 2021Review
- Common Pitfalls in Ewing Sarcoma and Desmoplastic Small Round Cell Tumor Diagnosis Seen in a Study of 115 Cases.Medical sciences (Basel, Switzerland) · 2021Article
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that affects the soft tissue and bones in middle-aged and elderly adults. Its diagnosis can be challenging, with the differential diagnoses including a wide variety of mesenchymal tumors. The line of differentiation of extraskeletal myxoid chondrosarcoma has been controversial, but recent evidence suggests a neuroendocrine phenotype. INSM1 is a zinc-finger transcription factor that plays a pivotal role in neuroendocrine differentiation, and has been proposed as a promising immunohistochemical marker of neuroendocrine carcinoma. The aim of this study was to determine the prevalence of INSM1 expression in extraskeletal myxoid chondrosarcoma and to understand its significance in sarcoma diagnosis. We immunostained the representative sections of 31 NR4A3-rearranged extraskeletal myxoid chondrosarcomas and 187 histological mimics. Nuclear staining of moderate or higher intensity in at least 5% of tumor cells was considered positive. Twenty-eight of the 31 extraskeletal myxoid chondrosarcomas (90%) were positive for INSM1, providing strong evidence for neuroendocrine differentiation. The staining was diffuse (>50%) in 17 cases, with most immunopositive tumors showing at least focal strong expression. The INSM1 staining extent was not correlated with cytomorphology, synaptophysin expression, or fusion types (EWSR1 vs non-EWSR1). In contrast, INSM1 expression was negative in 94% of the 187 other mesenchymal tumors. INSM1-positive mimics comprised a small subset of chordoma (1 of 10), soft tissue myoepithelioma (1 of 20), ossifying fibromyxoid tumor (3 of 10), and Ewing sarcoma (3 of 10), among other tumor types. The majority of these cases showed labeling in <25% of the tumor cells. Although not entirely sensitive or specific, INSM1 could be a potential marker for the diagnosis of extraskeletal myxoid chondrosarcoma when molecular genetic access is limited.
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