ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2018
Clinicopathologic characteristics of poorly differentiated chordoma.
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 64 papers, 2 of them syntheses that pooled it.
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Who cites it
64 citing papers in PubMed, 2 syntheses or guidelines pooled it, 134 citations in OpenAlex.
- Pooled it
- [Update and interpretation of 2021 National Comprehensive Cancer Network (NCCN) "Clinical Practice Guidelines for Bone Tumors"].Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery · 2021Guideline
- Tazemetostat, an EZH2 inhibitor, in solid tumors harboring SWI/SNF alterations: a phase II basket study.Nature communications · 2026Trial
- Atypical notochordal cell tumors: an alternative diagnosis when diagnostic criteria are not straightforward : Short title: atypical notochordal cell tumor.Virchows Archiv : an international journal of pathology · 2026Article
- Epithelioid Sarcoma of the Oral Cavity: A Multi-institutional Clinicopathologic and Immunophenotypic Characterization of Five Cases and Comprehensive Literature Review.Head and neck pathology · 2026Review
- Institutional experience and pooled survival analysis of chemotherapy based multimodal management in poorly differentiated chordoma.Discover oncology · 2026Article
- A patient-derived organoid model identifies TP53-dependent gemcitabine sensitivity in spinal chordoma.Discover oncology · 2026Article
- INI-1 Loss with brachyury positivity unmasking a cervical poorly differentiated chordoma initially diagnosed as epithelioid sarcoma: a case report.Frontiers in surgery · 2026Article
- Comprehensive profiling of paediatric chordoma: poorly differentiated and conventional subtypes.Brain communications · 2026Article
- A clinical analysis of 19 cases of chordomas with poor differentiation treated using diverse therapeutic modalities.Discover oncology · 2025Article
- Poorly Differentiated Aggressive Sacral Chordoma With Upfront Lung Metastases in a Child-A Case Report.Clinical case reports · 2025Article
- Epigenetic roles of chromatin remodeling complexes in bone biology and the pathogenesis of bone‑related disease (Review).International journal of molecular medicine · 2025Review
- SMARCB1 orchestrates cellular plasticity and oncogenic pathways in typical and chondroid chordomas.Scientific reports · 2025Article
- Characteristics and survival outcomes in pediatric patients with spinal chordomas: insights from the National Cancer Database and review of the literature.Journal of neuro-oncology · 2025Review
- Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature.North American Spine Society journal · 2024Article
- Poorly differentiated chordoma: recognising this complex and rare aggressive tumour with characteristic immunohistochemical profile.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Article
- Chordoma: Genetics and Contemporary Management.International journal of molecular sciences · 2024Review
- Poorly differentiated extra-axial extraskeletal chordoma diagnosed by methylation profiling: case report and analysis of brachyury expression in SWI/SNF-deficient tumors.Virchows Archiv : an international journal of pathology · 2024Article
- SMARCB1-Deficient Skull Base Chondrosarcoma with 12p Duplication Presenting as Somatic-Type Malignancy Arising from Metastatic Seminoma.Head and neck pathology · 2024Article
4 more citing papers are in PubMed but not listed here.
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Chordoma is a rare malignant tumor of bone with high morbidity and mortality. Recently, aggressive pediatric poorly differentiated chordoma with SMARCB1 loss has been described. This study summarizes the clinicopathologic features of poorly differentiated chordoma with SMARCB1 loss in the largest series to date. A search of records between 1990-2017 at MGH identified 19 patients with poorly differentiated chordoma. Immunohistochemical stains were evaluated. Kaplan-Meier survival statistics and log-rank (Mantel Cox) tests compared survival with other subtypes. The patients (n = 19) were diagnosed at a median age of 11 years (range: 1-29). Tumors arose in the skull base and clivus (n = 10/19; 53%); cervical spine (n = 6/19; 32%); and sacrum or coccyx (n = 3/19; 16%). The clinical stage of these patients (AJCC 7e) was stage 2A (n = 7/16; 44%); stage 2B (n = 6/16; 38%); stage 4A (n = 1/16; 6%); and stage 4B (n = 2/16; 13%). The tumors were composed of sheets of epithelioid cells with nuclear pleomorphism, abundant eosinophilic cytoplasm, and increased mitoses. Tumors were positive for cytokeratin (n = 18/18; 100%) and brachyury (n = 18/18; 100%). Patients were treated with a combination of excision, radiation therapy, and chemotherapy. No difference in overall survival, progression free survival, local control time, and metastasis free survival was identified between poorly differentiated chordoma of the skull base and of the spine. Compared to other chordoma subtypes, poorly differentiated chordoma has a significantly decreased mean overall survival after stratification by site (p = 0.037). Pediatric poorly differentiated chordoma has a distinct clinical and immunohistochemical profile, with characteristic SMARCB1 loss and decreased survival compared to conventional/chondroid chordoma. Recognition of this subtype is important because these malignancies should be treated aggressively with multimodality therapy.
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