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Malignant potential of neuroendocrine microtumor of the pancreas harboring high-grade transformation: lesson learned from a patient with von Hippel-Lindau syndrome
Jongwon Lee, Kyung Jin Lee, Dae Wook Hwang, Seung-Mo Hong
J Pathol Transl Med. 2024;58(2):91-97.   Published online March 13, 2024
DOI: https://doi.org/10.4132/jptm.2024.02.13
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  • 3 Web of Science
  • 3 Crossref
AbstractAbstract PDF
Pancreatic neuroendocrine microtumor (PNEMT) is a neuroendocrine tumor (NET) < 0.5 cm in diameter, and it is considered benign. We report a PNEMT with high-grade transformation (HGT). A man in his 60s with von Hippel-Lindau syndrome underwent surgical resection of a NET. A second sub-centimeter nodule with a nodule-in-nodule pattern was discovered. The 0.4 cm outer nodule contained clear columnar cells with round nuclei and indistinct nucleoli, while the 0.1 cm inner nodule had eosinophilic cells with an increased nuclear to cytoplasmic ratio, vesicular nuclei, and prominent nucleoli. Tumor cells in the outer and inner nodules were synaptophysin and chromogranin positive. Only the inner nodule was p53 positive, while the outer nodule was exclusively positive for carbonic anhydrase 9 and vimentin. The Ki-67 labeling indices for the outer and inner nodules were 2.1% (grade 1) and 44.3% (grade 3), respectively. This nodule was determined to be a PNEMT with HGT. Our findings suggest that a PNEMT may not always be benign and can undergo HGT.

Citations

Citations to this article as recorded by  
  • Decoding Pancreatic Neuroendocrine Tumors: Molecular Profiles, Biomarkers, and Pathways to Personalized Therapy
    Linda Galasso, Federica Vitale, Gabriele Giansanti, Giorgio Esposto, Raffaele Borriello, Irene Mignini, Alberto Nicoletti, Lorenzo Zileri Dal Verme, Antonio Gasbarrini, Maria Elena Ainora, Maria Assunta Zocco
    International Journal of Molecular Sciences.2025; 26(16): 7814.     CrossRef
  • Pancreatic neuroendocrine microtumors in the elderly: A retrospective study using cadaveric pancreatic tissue
    Ting Yang, Ke Ren, Xiang-Quan Chen, Taku Toriumi, Yutaro Natsuyama, Jun Li, Aoi Sukeda, Toshitaka Nagao, Shuang-Qin Yi
    World Journal of Gastrointestinal Oncology.2025;[Epub]     CrossRef
  • Molecular Basis of Pancreatic Neuroendocrine Tumors
    Alesia Maluchenko, Denis Maksimov, Zoia Antysheva, Julia Krupinova, Ekaterina Avsievich, Olga Glazova, Natalia Bodunova, Nikolay Karnaukhov, Ilia Feidorov, Diana Salimgereeva, Mark Voloshin, Pavel Volchkov
    International Journal of Molecular Sciences.2024; 25(20): 11017.     CrossRef

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