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Aims: Thymic epithelial tumours (TET), including thymomas and thymic carcinomas and thymic neuroendocrine neoplasms, are malignant neoplasms that can be associated with morbidity and mortality. Recently, an updated version of the World Health Organization (WHO) Classification of Thoracic Tumours 5th Edition, 2021 has been released, which included various changes to the classification of these neoplasms. In addition, in 2017 the Union for International Cancer Control (UICC) / American Joint Committee on Cancer (AJCC) published the 8th Edition Staging Manual which, for the first time, includes a TNM staging that is applicable to thymomas, thymic carcinomas, and thymic neuroendocrine neoplasms.
Methods And Results: To standardize reporting of resected TET and thymic neuroendocrine neoplasms the accrediting bodies updated their reporting protocols. The International Collaboration on Cancer Reporting (ICCR), which represents a collaboration between various National Associations of Pathology, updated its 2017 histopathology reporting guide on TET and thymic neuroendocrine neoplasms accordingly. This report will highlight important changes in the reporting of TET and thymic neuroendocrine neoplasms based on the 2021 WHO, emphasize the 2017 TNM staging, and also comment on the rigour and various uncertainties for the pathologist when trying to follow that staging.
Conclusion: The ICCR dataset provides a comprehensive, standardized template for reporting of resected TET and thymic neuroendocrine neoplasms.
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http://dx.doi.org/10.1111/his.15047 | DOI Listing |
Lung Cancer
August 2025
Division of Thoracic Surgery, Keio University School of Medicine, Tokyo, Japan.
Introduction: Thymic neuroendocrine tumors (TNETs) are rare malignancies characterized by aggressive clinical behavior and limited therapeutic options. In small cell lung cancer (SCLC), molecular subtypes based on the expression of lineage-defining transcription factors (TFs)-ASCL1, NEUROD1, POU2F3, and YAP1-have been proposed. However, the TF landscape of TNETs remains poorly defined.
View Article and Find Full Text PDFGenes Chromosomes Cancer
August 2025
Department of Pathology, Nanfang Hospital, School of Basic Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China.
Mastermind-like transcriptional coactivator (MAML) gene fusions have been documented in Thymic Epithelial Tumors (TETs). Specifically, lysine methyltransferase 2A (KMT2A)::MAML2 gene fusions are associated with type B2 and B3 thymomas. Here, we report for the first time a young patient with invasive type B3 thymoma harboring a novel KMT2A::MAML3 gene fusion.
View Article and Find Full Text PDFMed Sci (Basel)
July 2025
Department of Surgical Sciences, Uppsala University, 752 36 Uppsala, Sweden.
: Atypical carcinoid of the thymus is an exceptionally rare neuroendocrine tumor originating from neuroendocrine cells within the thymus. These tumors often present with no symptoms or with nonspecific clinical signs, making early diagnosis particularly challenging. Despite their rarity, atypical carcinoids are clinically significant due to their aggressive nature and relatively poor prognosis.
View Article and Find Full Text PDFEur J Surg Oncol
August 2025
Institute of Systems Molecular and Integrative Biology, University of Liverpool, Liverpool, UK; Department of Paediatric Surgery, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Electronic address:
Neuroendocrine tumors (NETs) are rare in the pediatric age group with studies reportedly showing increased incidence in females. The appendix appears to be the most common tumor site, but NETs can arise almost anywhere in the body. Due to rarity, comprehensive reports detailing outcomes are sparse and often based on limited index case numbers.
View Article and Find Full Text PDFEndocr Pathol
July 2025
Department of Pathology, University Hospitals Cleveland, Case Western Reserve University, Institute of Pathology, 11100 Euclid Avenue, Room 204, Cleveland, OH, USA.
Delta-like protein 3 (DLL3), a Notch ligand, has been identified in high-grade small- and large-cell lung carcinomas and prostate neuroendocrine carcinomas (NECs). SEZ6 (Seizure-related 6 homolog), a membrane-associated protein, has also been identified neuroendocrine neoplasms (NENs). Both DLL3 and SEZ6 are targets of novel antibody-drug conjugates (ADCs).
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