ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2015
Pancreatic undifferentiated rhabdoid carcinoma: KRAS alterations and SMARCB1 expression status define two subtypes.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
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50 citing papers in PubMed, 111 citations in OpenAlex.
- SWI/SNF Complex-Deficient Undifferentiated Carcinoma of the Pancreas: Clinicopathologic and Genomic Analysis.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2024Trial
- Pancreatic ductal adenocarcinoma and its subtypes: clinical relevance of histopathology and molecular characterization, integrating the key updates of the 2026 WHO classification.Virchows Archiv : an international journal of pathology · 2026Review
- Changes in the 6th edition of the World Health Organization classification of tumours of the digestive system.Histopathology · 2026Review
- INI1-Deficient malignancy involving the pancreas and jejunum: Expanding the clinical spectrum.Radiology case reports · 2026Article
- Clinicopathologic features of SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma: A case report and review of literature.World journal of gastrointestinal oncology · 2026Article
- Decoding UTROSCT heterogeneity: systematic clinicopathological evaluation combined with molecular profiling.The journal of pathology. Clinical research · 2026Article
- Rare pancreatic ductal adenocarcinoma variants and other malignant epithelial tumors: a comprehensive clinical and radiologic review.Japanese journal of radiology · 2025Review
- Clinicogenomic landscape of pancreatic adenocarcinoma identifies KRAS mutant dosage as prognostic of overall survival.Nature medicine · 2025Article
- Characterization of undifferentiated carcinomas of the pancreas with and without osteoclast-like giant cells.JNCI cancer spectrum · 2025Article
- Metastatic INI-1-deficient undifferentiated lung cancer with EGFR 19del mutation identified in pleural effusion: a case report and review of the literature.Frontiers in oncology · 2025Article
- Histological variants of pancreatic ductal adenocarcinoma: a survival analysis.Langenbeck's archives of surgery · 2024Article
- Sarcomatoid carcinoma of the pancreas (Review).Oncology letters · 2024Review
- SMARCB1/INI1-deficient undifferentiated tumour of the thorax: a case report and review of the literature.Pathologica · 2024Review
- [Histopathologic diagnosis of solid and cystic pancreatic lesions with a focus on ductal adenocarcinoma : A vademecum for daily practice].Pathologie (Heidelberg, Germany) · 2024Review
- A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas?Therapeutic advances in medical oncology · 2024Review
- Article
- Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells: a case report.Journal of medical case reports · 2023Article
- Comprehensive review of undifferentiated carcinoma of the pancreas: from epidemiology to treatment.Japanese journal of clinical oncology · 2023Review
- The Dual Role of Necroptosis in Pancreatic Ductal Adenocarcinoma.International journal of molecular sciences · 2023Review
- Diagnosis of thoracic SMARCA4-deficient undifferentiated tumor in cytology.Cancer cytopathology · 2023Article
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Authors and funding
8 authors at 4 institutions in 2 countries.
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No grant is acknowledged in the PubMed record.
Abstract
Pancreatic undifferentiated carcinoma is a heterogeneous group of neoplasms, including pleomorphic giant cell, sarcomatoid, round cell, and rhabdoid carcinomas, the molecular profiles of which have so far been insufficiently characterized. We studied 14 undifferentiated carcinomas with prominent rhabdoid cells, occurring as advanced tumors in seven females and seven males aged 44-96 years (mean: 65 years). Histologically, 10 tumors qualified as pleomorphic giant cell and 4 as monomorphic anaplastic carcinomas. A glandular component, either in the primary or in the metastases, was seen in 5 out of 14 tumors (4 out of 10 pleomorphic giant cell and 1 out of 4 monomorphic anaplastic subtypes, respectively). Osteoclast-like giant cells were absent. Immunohistochemistry revealed coexpression of cytokeratin and vimentin, and loss of membranous β-catenin and E-cadherin staining in the majority of cases. Nuclear SMARCB1 (INI1) expression was lost in 4 out of 14 cases (28%), representing all 4 tumors of the monomorphic anaplastic subtype. FISH and mutation testing of KRAS revealed KRAS amplification in 5 out of 13 (38%) and exon 2 mutations in 6 out of 11 (54%) successfully analyzed cases. A strong correlation was found between KRAS alterations (mutation and/or copy number changes) and intact SMARCB1 expression (7 out of 8; 87%). On the other hand, loss of SMARCB1 expression correlated with the absence of KRAS alterations (3 out of 5 cases; 60%). The results suggest that rhabdoid phenotype in pancreatic undifferentiated rhabdoid carcinomas has a heterogeneous genetic background. SMARCB1 loss is restricted to the anaplastic monomorphic subtype and correlates with the absence of KRAS alterations, whereas the pleomorphic giant cell subtype is characterized by KRAS alterations and intact SMARCB1 expression. Recognition and appropriate subtyping of these rare variants might become necessary for future therapeutic strategies.
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