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Pancreatic High-Grade Neuroendocrine Neoplasms in the Korean Population: A Multicenter Study
Haeryoung Kim, Soyeon An, Kyoungbun Lee, Sangjeong Ahn, Do Youn Park, Jo-Heon Kim, Dong-Wook Kang, Min-Ju Kim, Mee Soo Chang, Eun Sun Jung, Joon Mee Kim, Yoon Jung Choi, So-Young Jin, Hee Kyung Chang, Mee-Yon Cho, Yun Kyung Kang, Myunghee Kang, Soomin Ahn, Youn Wha Kim, Seung-Mo Hong, on behalf of the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists
Cancer Res Treat. 2020;52(1):263-276.   Published online July 12, 2019
DOI: https://doi.org/10.4143/crt.2019.192
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
The most recent 2017 World Health Organization (WHO) classification of pancreatic neuroendocrine neoplasms (PanNENs) has refined the three-tiered 2010 scheme by separating grade 3 pancreatic neuroendocrine tumors (G3 PanNETs) from poorly differentiated pancreatic neuroendocrine carcinomas (PanNECs). However, differentiating between G3 Pan- NETs and PanNECs is difficult in clinical practice.
Materials and Methods
Eighty-two surgically resected PanNENs were collected from 16 institutions and reclassified according to the 2017 WHO classification based on the histological features and proliferation index (mitosis and Ki-67). Immunohistochemical stains for ATRX, DAXX, retinoblastoma, p53, Smad4, p16, and MUC1 were performed for 15 high-grade PanNENs.
Results
Re-classification resulted in 20 G1 PanNETs (24%), 47 G2 PanNETs (57%), eight G3 well-differentiated PanNETs (10%), and seven poorly differentiated PanNECs (9%). PanNECs showed more frequent diffuse nuclear atypia, solid growth patterns and apoptosis, less frequent organoid growth and regular vascular patterns, and absence of low-grade PanNET components than PanNETs. The Ki-67 index was significantly higher in PanNEC (58.2%± 15.1%) compared to G3 PanNET (22.6%±6.1%, p < 0.001). Abnormal expression of any two of p53, p16, MUC1, and Smad4 could discriminate PanNECs from G3 PanNETs with 100% specificity and 87.5% sensitivity.
Conclusion
Histological features supporting the diagnosis of PanNECs over G3 PanNETs were the absence of a low-grade PanNET component in the tumor, the presence of diffuse marked nuclear atypia, solid growth pattern, frequent apoptosis and markedly increased proliferative activity with homogeneous Ki-67 labeling. Immunohistochemical stains for p53, p16, MUC1, and Smad4 may be helpful in distinguishing PanNECs from G3 PanNETs in histologically ambiguous cases, especially in diagnostic practice when only small biopsied tissues are available.

Citations

Citations to this article as recorded by  
  • Treatment status, survival and gene expression analysis of large-cell neuroendocrine lung carcinoma: a real-world study in China
    Fei Qi, Minghang Zhang, Yi Han, Juan Du, Hongjie Yang, Hongmei Zhang, Yong Zhang, Tongmei Zhang
    Therapeutic Advances in Medical Oncology.2025;[Epub]     CrossRef
  • Pancreatic neuroendocrine neoplasms (pNENs): Genetic and environmental biomarkers for risk of occurrence and prognosis
    Matteo Tacelli, Manuel Gentiluomo, Paolo Biamonte, Justo P. Castano, Maja Cigrovski Berković, Mauro Cives, Sanja Kapitanović, Ilaria Marinoni, Sonja Marinovic, Ilias Nikas, Lenka Nosáková, Sergio Pedraza-Arevalo, Eleonora Pellè, Aurel Perren, Jonathan Str
    Seminars in Cancer Biology.2025; 112: 112.     CrossRef
  • 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
    Journal of Pathology and Translational Medicine.2024; 58(2): 91.     CrossRef
  • Rapid Evolution of Metastases in Patients with Treated G3 Neuroendocrine Tumors Associated with NEC-Like Transformation and TP53 Mutation
    Atsuko Kasajima, Nicole Pfarr, Eva-Maria Mayr, Ayako Ura, Elisa Moser, Alexander von Werder, Abbas Agaimy, Marianne Pavel, Günter Klöppel
    Endocrine Pathology.2024; 35(4): 313.     CrossRef
  • The Complex Histopathological and Immunohistochemical Spectrum of Neuroendocrine Tumors—An Overview of the Latest Classifications
    Ancuța-Augustina Gheorghișan-Gălățeanu, Andreea Ilieșiu, Ioana Maria Lambrescu, Dana Antonia Țăpoi
    International Journal of Molecular Sciences.2023; 24(2): 1418.     CrossRef
  • All Together Now
    Pari Jafari, Aliya N. Husain, Namrata Setia
    Surgical Pathology Clinics.2023; 16(1): 131.     CrossRef
  • A systematic review of therapeutic strategies in gastroenteropancreatic grade 3 neuroendocrine tumors
    Mauro D. Donadio, Ângelo B. Brito, Rachel P. Riechelmann
    Therapeutic Advances in Medical Oncology.2023;[Epub]     CrossRef
  • MicroRNAs associated with postoperative outcomes in patients with limited stage neuroendocrine carcinoma of the esophagus
    Tomoyuki Okumura, Tsutomu Fujii, Kenji Terabayashi, Takashi Kojima, Shigeru Takeda, Tomomi Kashiwada, Kazuhiro Toriyama, Susumu Hijioka, Tatsuya Miyazaki, Miho Yamamoto, Shunsuke Tanabe, Yasuhiro Shirakawa, Masayuki Furukawa, Yoshitaka Honma, Isamu Hoshin
    Oncology Letters.2023;[Epub]     CrossRef
  • The association between jaundice and poorly differentiated pancreatic neuroendocrine neoplasms (Ki67 index > 55.0%)
    Yongkang Liu, Jiangchuan Wang, Hao Zhou, Zicheng Wei, Jianhua Wang, Zhongqiu Wang, Xiao Chen
    BMC Gastroenterology.2023;[Epub]     CrossRef
  • An analysis of 130 neuroendocrine tumors G3 regarding prevalence, origin, metastasis, and diagnostic features
    Atsuko Kasajima, Björn Konukiewitz, Anna Melissa Schlitter, Wilko Weichert, Günter Klöppel
    Virchows Archiv.2022; 480(2): 359.     CrossRef
  • An update on genetically engineered mouse models of pancreatic neuroendocrine neoplasms
    Tiago Bordeira Gaspar, José Manuel Lopes, Paula Soares, João Vinagre
    Endocrine-Related Cancer.2022; 29(12): R191.     CrossRef
  • Solid pancreatic masses in children: A review of current evidence and clinical challenges
    Kelli N. Patterson, Andrew T. Trout, Archana Shenoy, Maisam Abu-El-Haija, Jaimie D. Nathan
    Frontiers in Pediatrics.2022;[Epub]     CrossRef
  • Neuroendocrine Carcinomas with Atypical Proliferation Index and Clinical Behavior: A Systematic Review
    Tiziana Feola, Roberta Centello, Franz Sesti, Giulia Puliani, Monica Verrico, Valentina Di Vito, Cira Di Gioia, Oreste Bagni, Andrea Lenzi, Andrea M. Isidori, Elisa Giannetta, Antongiulio Faggiano
    Cancers.2021; 13(6): 1247.     CrossRef
  • Risk of cancer in patients with recurrent aphthous stomatitis in Korea
    Ki Jin Kwon, Su Jin Jeong, Young-Gyu Eun, In Hwan Oh, Young Chan Lee
    Medicine.2021; 100(16): e25628.     CrossRef
  • Digestive Well-Differentiated Grade 3 Neuroendocrine Tumors: Current Management and Future Directions
    Anna Pellat, Anne Ségolène Cottereau, Lola-Jade Palmieri, Philippe Soyer, Ugo Marchese, Catherine Brezault, Romain Coriat
    Cancers.2021; 13(10): 2448.     CrossRef
  • Neuroendocrine Carcinomas of the Digestive Tract: What Is New?
    Anna Pellat, Anne Ségolène Cottereau, Benoit Terris, Romain Coriat
    Cancers.2021; 13(15): 3766.     CrossRef
  • Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation
    Lisa Qiu, Andrew T. Trout, Rama S. Ayyala, Sara Szabo, Jaimie D. Nathan, James I. Geller, Jonathan R. Dillman
    RadioGraphics.2021; 41(6): 1766.     CrossRef
  • CD56 Expression Is Associated with Biological Behavior of Pancreatic Neuroendocrine Neoplasms


    Xin Chen, Chuangen Guo, Wenjing Cui, Ke Sun, Zhongqiu Wang, Xiao Chen
    Cancer Management and Research.2020; Volume 12: 4625.     CrossRef
  • Prognostic and predictive factors on overall survival and surgical outcomes in pancreatic neuroendocrine tumors: recent advances and controversies
    Lingaku Lee, Tetsuhide Ito, Robert T Jensen
    Expert Review of Anticancer Therapy.2019; 19(12): 1029.     CrossRef
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Incremental Role of Pancreatic Magnetic Resonance Imaging after Staging Computed Tomography to Evaluate Patients with Pancreatic Ductal Adenocarcinoma
Hye Jin Kim, Mi-Suk Park, Jin Yong Lee, Kyunghwa Han, Yong Eun Chung, Jin-Young Choi, Myeong-Jin Kim, Chang Moo Kang
Cancer Res Treat. 2019;51(1):24-33.   Published online February 5, 2018
DOI: https://doi.org/10.4143/crt.2017.404
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
The purpose of this study was to investigate the impact of contrast enhanced pancreatic magnetic resonance imaging (MRI) in resectability and prognosis evaluation after staging computed tomography (CT) in patients with pancreatic ductal adenocarcinoma (PDA).
Materials and Methods
From January 2005 to December 2012, 298 patients were diagnosed to have potentially resectable stage PDA on CT. Patients were divided into CT+MR (patients underwent both CT and MRI; n=216) and CT only groups (n=82). Changes in resectability staging in the CT+MR group were evaluated. The overall survival was compared between the two groups. The recurrence-free survival and median time to liver metastasis after curative surgery were compared between the two groups.
Results
Staging was changed from resectable on CT to unresectable state on MRI in 14.4% of (31 of 216 patients) patients of the CT+MR group. The overall survival and recurrence-free survival rates were not significantly different between the two groups (p=0.162 and p=0.721, respectively). The median time to liver metastases after curative surgery in the CT+MR group (9.9 months) was significantly longer than that in the CT group (4.2 months) (p=0.011).
Conclusion
Additional MRI resulted in changes of resectability and treatment modifications in a significant proportion of patients who have potentially resectable state at CT and in prolonged time to liver metastases in patients after curative surgery. Additional MRI to standard staging CT can be recommended for surgical candidates of PDA.

Citations

Citations to this article as recorded by  
  • Pancreatic ductal adenocarcinoma: the latest on diagnosis, molecular profiling, and systemic treatments
    Doaa Bugazia, Ebtesam Al-Najjar, Abdullah Esmail, Saifudeen Abdelrahim, Karen Abboud, Adham Abdelrahim, Godsfavour Umoru, Hashem A. Rayyan, Ala Abudayyeh, Ala-Eddin Al Moustafa, Maen Abdelrahim
    Frontiers in Oncology.2024;[Epub]     CrossRef
  • S3-Leitlinie Exokrines Pankreaskarzinom – Version 3.1
    Thomas Seufferlein, Julia Mayerle, Stefan Boeck, Thomas Brunner, Thomas J. Ettrich, Lars Grenacher, Thomas Mathias Gress, Thilo Hackert, Volker Heinemann, Angelika Kestler, Marianne Sinn, Andrea Tannapfel, Ulrich Wedding, Waldemar Uhl
    Zeitschrift für Gastroenterologie.2024; 62(10): e874.     CrossRef
  • S3-Leitlinie Exokrines Pankreaskarzinom – Version 3.1
    Thomas Seufferlein, Julia Mayerle, Stefan Boeck, Thomas Brunner, Thomas J. Ettrich, Lars Grenacher, Thomas Mathias Gress, Thilo Hackert, Volker Heinemann, Angelika Kestler, Marianne Sinn, Andrea Tannapfel, Ulrich Wedding, Waldemar Uhl
    Zeitschrift für Gastroenterologie.2024; 62(10): 1724.     CrossRef
  • Additional MRI for initial M-staging in pancreatic cancer: a cost-effectiveness analysis
    Felix G. Gassert, Sebastian Ziegelmayer, Johanna Luitjens, Florian T. Gassert, Fabian Tollens, Johann Rink, Marcus R. Makowski, Johannes Rübenthaler, Matthias F. Froelich
    European Radiology.2022; 32(4): 2448.     CrossRef
  • Diagnostik beim Pankreaskarzinom – Update in der neuen Leitlinie
    L. Grenacher, M. Juchems, A. G. Schreyer, J. Wessling, K. I. Ringe, W. Uhl, J. Mayerle, T. Seufferlein
    Der Chirurg.2022; 93(5): 429.     CrossRef
  • Pancreatic adenocarcinoma: imaging techniques for diagnosis and management
    Jawaad Farrukh, Ravivarma Balasubramaniam, Anitha James, Sharan S Wadhwani, Raneem Albazaz
    British Journal of Hospital Medicine.2022; 83(5): 1.     CrossRef
  • S3-Leitlinie zum exokrinen Pankreaskarzinom – Kurzversion 2.0 – Dezember 2021, AWMF-Registernummer: 032/010OL
    Thomas Seufferlein, Julia Mayerle, Stefan Böck, Thomas Brunner, Thomas J. Ettrich, Lars Grenacher, Thomas Mathias Gress, Thilo Hackert, Volker Heinemann, Angelika Kestler, Marianne Sinn, Andrea Tannapfel, Ulrich Wedding, Waldemar Uhl
    Zeitschrift für Gastroenterologie.2022; 60(06): 991.     CrossRef
  • S3-Leitlinie zum exokrinen Pankreaskarzinom – Langversion 2.0 – Dezember 2021 – AWMF-Registernummer: 032/010OL
    Thomas Seufferlein, Julia Mayerle, Stefan Böck, Thomas Brunner, Thomas J. Ettrich, Lars Grenacher, Thomas Mathias Gress, Thilo Hackert, Volker Heinemann, Angelika Kestler, Marianne Sinn, Andrea Tannapfel, Ulrich Wedding, Waldemar Uhl
    Zeitschrift für Gastroenterologie.2022; 60(11): e812.     CrossRef
  • The Role of Imaging in Current Treatment Strategies for Pancreatic Adenocarcinoma
    Hyungjin Rhee, Mi-Suk Park
    Korean Journal of Radiology.2021; 22(1): 23.     CrossRef
  • A Comparative Study of Survivor Outcomes between Preoperative Evaluation Using CT Alone and Combined CT and MRI in Patients with Pancreatic Ductal Adenocarcinoma
    Ji Eun Lee, Seong Hyun Kim, Soon Jin Lee, Seo-Youn Choi, Sunyoung Lee, Bo Ra Lee
    Journal of the Korean Society of Radiology.2021; 82(3): 638.     CrossRef
  • СT and MRI in the assessment of resectable and borderline resectable pancreatic tumors
    A. V. Kudryavtseva, S. S. Bagnenko, I. I. Dzidzawa, I. S. Zheleznyak, G. E. Trufanov, V. V. Ryazanov, V. A. Krasovskaya, A. B. Kotiv, A. D. Kazakov
    Annaly khirurgicheskoy gepatologii = Annals of HPB Surgery.2021; 26(1): 34.     CrossRef
  • European Cancer Organisation Essential Requirements for Quality Cancer Care (ERQCC): Pancreatic Cancer
    Stefano Partelli, Francesco Sclafani, Sorin Traian Barbu, Marc Beishon, Pierluigi Bonomo, Graça Braz, Filippo de Braud, Thomas Brunner, Giulia Martina Cavestro, Mirjam Crul, Maria Die Trill, Piero Ferollà, Ken Herrmann, Eva Karamitopoulou, Cindy Neuzillet
    Cancer Treatment Reviews.2021; 99: 102208.     CrossRef
  • ISOlation Procedure vs. conventional procedure during Distal Pancreatectomy (ISOP-DP trial): study protocol for a randomized controlled trial
    Ken-ichi Okada, Manabu Kawai, Seiko Hirono, Masayuki Sho, Masaji Tani, Ippei Matsumoto, Suguru Yamada, Ryosuke Amano, Hirochika Toyama, Yo-ichi Yamashita, Takeshi Gocho, Kazuto Shibuya, Minako Nagai, Hiromitsu Maehira, Keiko Kamei, Go Ohira, Yoshihiro Shi
    Trials.2021;[Epub]     CrossRef
  • Management of Primary Squamous Cell Carcinoma of the Pancreas: A Case Report
    Danling Guo, Chao Chen, Sangying Lv, Guanzuan Wu, Wei Shi, Huaifeng Li, Hongjie Hu
    Frontiers in Surgery.2021;[Epub]     CrossRef
  • Tumor-Specific miRNA Signatures in Combination with CA19-9 for Liquid Biopsy-Based Detection of PDAC
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    International Journal of Molecular Sciences.2021; 22(24): 13621.     CrossRef
  • Imaging and Management of Pancreatic Cancer
    Mariya Kobi, Gregory Veillette, Roshni Narurkar, David Sadowsky, Viktoriya Paroder, Chaitanya Shilagani, Anthony Gilet, Milana Flusberg
    Seminars in Ultrasound, CT and MRI.2020; 41(2): 139.     CrossRef
  • DUPLICATE: Imaging and Management of Pancreatic Cancer
    Mariya Kobi, Gregory Veillette, Roshni Narurkar, David Sadowsky, Viktoriya Paroder, Chaitanya Shilagani, Anthony Gilet, Milana Flusberg
    Seminars in Ultrasound, CT and MRI.2019;[Epub]     CrossRef
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  • 319 Download
  • 18 Web of Science
  • 17 Crossref
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Prognosis of Pancreatic Cancer Patients with Synchronous or Metachronous Malignancies from Other Organs Is Better than Those with Pancreatic Cancer Only
Su-Jin Shin, Hosub Park, You-Na Sung, Changhoon Yoo, Dae Wook Hwang, Jin-hong Park, Kyu-pyo Kim, Sang Soo Lee, Baek-Yeol Ryoo, Dong-Wan Seo, Song Cheol Kim, Seung-Mo Hong
Cancer Res Treat. 2018;50(4):1175-1185.   Published online December 20, 2017
DOI: https://doi.org/10.4143/crt.2017.494
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
Pancreatic cancer associated double primary tumors are rare and their clinicopathologic characteristics are not well elucidated.
Materials and Methods
Clinicopathologic factors of 1,352 primary pancreatic cancers with or without associated double primary tumors were evaluated.
Results
Of resected primary pancreatic cancers, 113 (8.4%) had associated double primary tumors, including 26 stomach, 25 colorectal, 18 lung, and 13 thyroid cancers. The median interval between the diagnoses of pancreatic cancer and associated double primary tumors was 0.5 months. Overall survival (OS) of pancreatic cancer patients with associated double primary tumors was longer than those with pancreatic cancer only (median, 23.1 months vs. 17.0 months, p=0.002). Patients whose pancreatic cancers were resected before the diagnosis of metachronous tumors had a better OS than patients whose pancreatic cancer resected after the diagnosis of metachronous tumors (48.9 months and 13.5 months, p=0.001) or those whose pancreatic cancers were resected synchronously with non-pancreas tumors (19.1 months, p=0.043). The OS of pancreatic cancer patients with stomach (33.9 months, p=0.032) and thyroid (117.8 months, p=0.049) cancers was significantly better than those with pancreas cancer only (17.0 months).
Conclusion
About 8% of resected pancreatic cancers had associated double primary tumors, and those from the colorectum, stomach, lung, and thyroid were common. Patients whose pancreatic cancer was resected before the diagnosis of metachronous tumors had better OS than those resected after the diagnosis of metachronous tumors or those resected synchronously.

Citations

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  • Laparoscopic Radical Total Gastrectomy and Pancreatosplenectomy for Synchronous Cancer of the Stomach and Pancreas
    Motoki Ebihara, Kentoku Fujisawa, Shusuke Haruta, Hironori Uruga, Masaki Ueno
    Cureus.2024;[Epub]     CrossRef
  • Synchronous primary multiple cancer: distal cholangiocarcinoma of the intrapancreatic common bile duct and intraductal papillary mucinous tumor associated with ductal adenocarcinoma of the pancreatic tail
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    Pirogov Russian Journal of Surgery.2024; (8): 57.     CrossRef
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    Oronzo Brunetti, Giuseppe Badalamenti, Simona De Summa, Angela Calabrese, Antonella Argentiero, Livia Fucci, Vito Longo, Domenico Galetta, Pia Maria Soccorsa Perrotti, Rosamaria Pinto, Daniela Petriella, Katia Danza, Stefania Tommasi, Francesco Leonetti,
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Case Reports
Multiple Cardiac Metastases from a Nonfunctioning Pancreatic Neuroendocrine Tumor
Yong Hyeok Choi, Hye-Suk Han, Sung-Nam Lim, Sang Yeub Lee, Ji Hae Koo, Ok-Jun Lee, Ki Hyeong Lee, Seung Taik Kim
Cancer Res Treat. 2013;45(2):150-154.   Published online June 30, 2013
DOI: https://doi.org/10.4143/crt.2013.45.2.150
AbstractAbstract PDFPubReaderePub
Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms, which most commonly metastasize to the liver. However, intrathoracic metastases from pNETs are encountered infrequently. This report describes a case of nonfunctioning pNET with multiple cardiac metastases. A 56-year-old male presented with a palpable abdominal mass that showed progressive enlargement. Findings on computed tomography (CT) of the abdomen revealed two relatively well-marginated inhomogeneous low-attenuation masses, one in the head of the pancreas and the other in the tail. Multiple enhancing masses in the left pericardium with myocardial involvement were observed on chest CT and transthoracic echocardiography. Needle biopsies were performed on the mass in the tail of the pancreas and the left ventricular apical pericardium; histologic examination by hematoxylin and eosin morphology and immunohistochemical staining showed pNET in both. This is the first report of pNET with multiple cardiac metastases to previously undescribed metastatic sites.

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  • Cardiac Neuroendocrine Tumor Metastases on68Ga-DOTATATE PET/CT: Identification and Prognostic Significance
    Hwan Lee, Ahmad S. Alhamshari, Vandan Patel, Abhijit Bhattaru, Chaitanya Rojulpote, Mahesh K. Vidula, Daniel A. Pryma, Paco E. Bravo
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    Agnieszka Styczeń, Mariusz Kozak, Marta Karaś-Głodek, Elżbieta Czekajska-Chehab, Andrzej Tomaszewski, Andrzej Wysokiński, Tomasz Zapolski
    Medicina.2021; 57(2): 107.     CrossRef
  • Cardiac Metastases in Patients with Neuroendocrine Tumours: Clinical Features, Therapy Outcomes, and Prognostic Implications
    Man Liu, Eleni Armeni, Shaunak Navalkissoor, Joseph Davar, Luke Sullivan, Charlotte Leigh, Luke Furtado O’Mahony, Aimee Hayes, Dalvinder Mandair, Jie Chen, Martyn Caplin, Christos Toumpanakis
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    Georgios Georgiopoulos, Panagiota Mitropoulou, Pier Giorgio Masci, Juerg Schwitter, Khan Tina, Voges Inga, Raisi Estabragh Zahra, Minguito Carazo Carlos, Thomson Ross
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Clear Cell Carcinoma of the Pancreas -A Case Report and Review of the Literature-
Hui-Young Lee, Dong-Gyu Lee, Kwangjin Chun, Seungkoo Lee, Seo-Young Song
Cancer Res Treat. 2009;41(3):175-181.   Published online September 29, 2009
DOI: https://doi.org/10.4143/crt.2009.41.3.175
AbstractAbstract PDFPubReaderePub

Most of the malignant neoplasms of the pancreas demonstrate features that are consistent with adenocarcinoma. According to the WHO classification, primary clear cell carcinoma of the pancreas is rare and it is classified as a "miscellaneous" carcinoma. In addition, there is not an adequate systematic overview that can demonstrate its true existence as a definable entity. We report here on an unusual case of primary pancreatic clear cell carcinoma, which is the first such reported case in Korea. A 66 year old woman presented with abdominal pain and significant weight loss over the previous three weeks. On the abdominal computed tomography (CT), we detected an abdominal mass involving the pancreas tail and liver, and clear cell carcinoma with rhabdoid feature was seen on the histologic evaluation. The tumor cells showed well defined cell membranes, clear cytoplasm and prominent cell boundaries. The immunohistochemical stains showed positive reactions to antibodies against pan-cytokeratin, cytokeratin 7, carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). On the other hand, there was a negative reaction for cytokeratin 20, chromogranin, synaptophysin, smooth muscle actin and HMB-45. She was diagnosed with a primary pancreatic clear cell carcinoma with hepatic metastasis and she received palliative gemcitabine chemotherapy. The patient died one month later of pancreatic cancer progression.

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  • Metastatic clear cell sarcoma of the pancreas: An overview
    Rachid Ait Addi
    World Journal of Clinical Cases.2024; 12(29): 6262.     CrossRef
  • Long-Term Survival After Curative Resection of a Primary Clear Cell Carcinoma of the Pancreas: A Report of a Case
    Helen Bolanaki, Christina Nikolaou, George Pappas Gogos, Anastasios J Karayiannakis
    Cureus.2024;[Epub]     CrossRef
  • Primary Clear Cell Carcinoma of the Pancreas: A Rare Case Report
    Yoo-Na Kang
    Diagnostics.2022; 12(9): 2046.     CrossRef
  • Primary Clear Cell Carcinoma of the Pancreas: A Systematic Review
    Toufic Tannous, Audrik L Perez Rodriguez, Andrew W Mak, Karim Tannous, Matthew Keating
    Cureus.2021;[Epub]     CrossRef
  • Two Cases of Rare Pancreatic Malignancies
    Wissam Hanayneh, Hiral Parekh, Garrett Fitzpatrick, Michael Feely, Thomas J. George, Jason S. Starr
    Journal of Pancreatic Cancer.2019; 5(1): 26.     CrossRef
  • Primary signet ring cell carcinoma of the pancreas: Cytopathology review of a rare entity
    Siba El Hussein, Samer N. Khader
    Diagnostic Cytopathology.2019; 47(12): 1314.     CrossRef
  • Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors
    Oronzo Brunetti, Giuseppe Aprile, Paolo Marchetti, Enrico Vasile, Andrea Casadei Gardini, Mario Scartozzi, Sandro Barni, Sara Delfanti, Fernando De Vita, Francesco Di Costanzo, Michele Milella, Chiara Alessandra Cella, Rossana Berardi, Ivana Cataldo, Aldo
    Pancreas.2018; 47(6): 759.     CrossRef
  • Hyalinizing clear cell carcinoma arising in the lower with cervical lymph node metastasis: Report of a case and review of literature
    Aya Hamao-Sakamoto, Jason Mull, Patrick Wang, John Randall Zuniga
    Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology.2014; 26(4): 585.     CrossRef
  • Canine Pancreatic Clear Acinar Cell Carcinoma Showing an Unusual Mucinous Differentiation
    S. Pavone, E. Manuali, C. Eleni, A. Ferrari, E. Bonanno, A. Ciorba
    Journal of Comparative Pathology.2011; 145(4): 355.     CrossRef
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A Case of Recurrent Solid Pseudopapillary Tumor of the Pancreas with Involvement of the Spleen and Kidney
Sang Eun Park, Nam Sook Park, Jae Min Chun, Nam Whan Park, Young Joon Yang, Gak Won Yun, Hyo Jin Lee, Hwan Jung Yun, Deog Yeon Jo, Kyu Sang Song, Samyong Kim
Cancer Res Treat. 2006;38(2):118-120.   Published online April 30, 2006
DOI: https://doi.org/10.4143/crt.2006.38.2.118
AbstractAbstract PDFPubReaderePub

Solid pseudopapillary tumor of the pancreas (SPTP) is a rare primary pancreatic tumor of an unknown etiology that is usually diagnosed in adolescent girls and young women. Most SPTPs are considered to be benign and only rarely metastasize. We report here on a 27-year old woman with recurrent SPTP with involvement of both the spleen and left kidney at the time of the initial diagnosis, and with aggressive behavior. In July 1995, she was admitted with abdominal discomfort and mass. She underwent exploratory laparotomy with distal pancrea tectomy, left nephrectomy and splenectomy, and was diagnosed with SPTP with invasion to both the spleen and left kidney. In June 2001, she again presented with abdominal pain and was diagnosed as having recurrence of the tumor. She underwent mass excision and omentectomy. Then she was lost to follow-up. In November 2005, she presented once again with an abdominal mass and was diagnosed with recurred SPTP, which formed a huge intraperitoneal mass with peritoneal seeding and the tumor showed multiple metastases in the liver. She is currently being treated conservatively.

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  • Clinical Pattern of Preoperative Positron Emission Tomography/Computed Tomography (PET/CT) Can Predict the Aggressive Behavior of Resected Solid Pseudopapillary Neoplasm of the Pancreas
    Ji-Su Kim, Emmanuel II-Uy Hao, Seoung-Yoon Rho, Ho-Kyoung Hwang, Woo-Jung Lee, Dong-Sub Yoon, Chang-Moo Kang
    Cancers.2021; 13(9): 2119.     CrossRef
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Original Articles
Gemcitabine and Infusional 5-Fluorouracil in Advanced Pancreatic Cancer: A Clinical Benefit Response-Oriented Phase II Study
Jung Hye Choi, Myung Ju Ahn, Seock Ah Im, Bong Seog Kim, Ho Suk Oh, Heung Woo Lee, Yeung Chul Mun, Chu Myung Seong, Soon Nam Lee, Young Yeul Lee, Il Young Choi, In Soon Kim
Cancer Res Treat. 2003;35(3):213-217.   Published online June 30, 2003
DOI: https://doi.org/10.4143/crt.2003.35.3.213
AbstractAbstract PDF
PURPOSE
Gemcitabine and 5-fluorouracil (5-FU) are two compounds with reproducible activity against advanced pancreatic carcinomas. To evaluate the activity and feasibility of this combination chemotherapy, a multi-institutional phase II study was performed. MATERIALS AND METHODS: Twenty patients (male: female 15: 5, median age: 60.5 years), with histologically verified locally advanced or metastatic pancreatic carcinomas, were enrolled between April 2000 and March 2002. Gemcitabine was administered by intravenous injection at the doses of 1, 000 mg/m2 on days 1, 8 and 15, and 5-FU 800 mg/m2/day, was given by continuous intravenous infusion on days 1~5. The treatment was repeated every 4 weeks. The clinical benefit response (CBR) was a composite of the pain, Karnofsky performance status and body weight change measurement.
RESULTS
Nineteen of the twenty patients were assessable for response. The median follow-up duration was 4.6 months (0.4~15.2 months). Five patients achieved a partial response and eight a stable disease. The overall response rate was 25.0%. The CBR was assessable in 12 patients. The overall CBR was 41.7% (5/12). The median survival of all the patients was 8.0 months. Grade 3~4 toxicities included neutropenia (9.3%) and thrombocytopenia (5.3%). CONCLUSION: This study suggested that gemcitabine, combined with infusional 5-FU, was well tolerated, and produced modest antitumor activity and symptomatic relief in advanced pancreatic cancer patients.
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Phase II Trial of Gemcitabine, UFT-E, Leucovorin Combination Chemotherapy in Advanced Pancreatic Adenocarcinoma
So Young Yoon, Kyong Hwa Park, Sang Chul Oh, Jae Hong Seo, Chul Won Choi, Byung Soo Kim, Jae Seon Kim, Chang Duck Kim, Sang Won Shin, Yeul Hong Kim, Jun Suk Kim
Cancer Res Treat. 2002;34(2):111-116.   Published online April 30, 2002
DOI: https://doi.org/10.4143/crt.2002.34.2.111
AbstractAbstract PDF
PURPOSE
To evaluate the efficacy and toxicity of a Gemcitabine, UFT-E, Leucovorin combination chemotherapy in the treatment of advanced pancreatic adenocarcinoma. PATIENTS AND METHODS: Patients <=70 years, with no prior chemotherapy and with bidimensionally measurable advanced pancreatic adenocarcinoma, ECOG performance status <=2, and adequate bone marrow, kidney, and liver function were eligible for this trial. Eligibility criteria for clinical benefit assessment were pain with at least a daily analgesic consumption of two nonsteroidal anti-inflammatory drugs or a Karnofsky performance status between 50 and 70. Treatment consisted of 1,000 mg/m2 of Gemcitabine on days 1, 8 and 15, repeated every 4 weeks, with UFT-E administered orally 500 mg-700 mg by body surface area (BSA). Leucovorin was administered 45 mg/day orally. Dosages of UFT-E and Leucovorin were divided and administered three times per day from day 1 to day 21. After 7 days of rest, UFT-E and Leucovorin were administered repeatedly.
RESULTS
Twenty-three patients were enrolled between April 1999 to April 2000. Eighty two cycles (median, four cycles) were delivered to all patients. The objective response rate was 15.8% in 19 assessable patients and 13.0% in the intent-to-treat population. Twelve patients (57.9%) displayed stable disease. Grade 3 or 4 neutropenia occurred in 30.4% of patients, nausea/vomiting in 8.3%, diarrhea in 4.3%, and mucositis in 4.3%. The median time to progression was 8 months. The median survival was 8 months in the assessable population and 6 months in the intent-to-treat population Clinical benefit was achieved in 11 (57.9%) of 19 assessable patients.
CONCLUSION
Gemcitabine, UFT-E, Leucovorin combination chemotherapy is a well-tolerated and safe regimen in cases of advanced pancreatic adenocarcinoma. Although the response rate is low, it shows a survival benefit and clinical benefit and deserves further evaluation in a phase III trial.

Citations

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  • Gemcitabine and oxaliplatin combination as first-line treatment for advanced pancreatic cancer: a multicenter phase II study
    Kyung Hee Lee, Min Kyoung Kim, Yeol Hong Kim, Baek Yeol Ryoo, Ho Yeong Lim, Hong Suk Song, Hoon Kyo Kim, Myung Ah Lee, Seock Ah Im, Heung Moon Chang, Jae Yong Cho, Dae Young Zang, Bong Seog Kim, Jun Suk Kim
    Cancer Chemotherapy and Pharmacology.2009; 64(2): 317.     CrossRef
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Pediatric Pancreatic Tumors
Dae Yeon Kim, Min Gew Choi, Ki Hong Kim, In One Kim, Sung Eun Jung, Seong Cheol Lee, Kwi Won Park, Woo Ki Kim
J Korean Cancer Assoc. 1999;31(4):830-835.
AbstractAbstract PDF
PURPOSE
Pancreatic tumors are relatively rare in children. Until now more than 150 cases have been reported in the English literature. In this paper, the authors report the tumors clinical characteristics and the results of surgery in eleven children.
MATERIALS AND METHODS
Eleven cases of pancreatic tumor pathologically verified at Seoul National University Children's Hospital between 1984 to 1998 were retrospectively analyzed. Four were boys and seven were girls, and their mean age at diagnosis was 7.7 (range, 2 13) years.
RESULTS
There were six solid and papillary epithelial neoplasms of the pancreas (SPENP) and five pancreatoblastomas. All children came to medical attention because of abdominal masses. Tumors ranged in size from 6.0X 5.0 cm to 10.5 x 8.0 cm. Eight tumors were located in head and three in tail. Complete excision was performed in eight cases (six in SPENP and two in pancreatoblastoma). Incomplete excision was performed in two cases of pancreatoblastoma. One patient with pancreatoblastoma had an unresectable tumor at the time of diagnosis and needle aspiration biopsy was done under the ultrasound guidance. No patient died during surgery. After a mean follow-up period of 4.1 years, all patients with SPENP were alive and there had been no recurrence. However, of two patients who received complete excision in pancreatoblastoma, one presented with liver metastasis at 4 months after operation and received chemotherapy, but died 6 months after operation. The other patient had local recurrence 1 year after operation. Reoperation and chemotherapy were performed and the child is now alive without evidence of disease montbs after the initial operation. All three patients with unresectable tumor died in spite of adjuvant radiotherapy and chemotherapy.
CONCLUSION
Pediatric pancreatic tumors comprise rare heterogenous groups of malignancies with their prognosis dependent upon adequate resection and pathologic classification. Complete resection of pancreatic tumors arising anywhere in the pancreas was recommended.
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Metastatic Papillary Cystic Neoplasm of Pancreas in Child - Report of a case -
Seung Tae Han, Kyung Rae Kim, Yong Hwa Moon, Young Chae Chu, Chuhl Joo Lyu, Byung Soo Kim
J Korean Cancer Assoc. 1990;22(3):600-607.
AbstractAbstract PDF
Papillary cystic neoplasm of the pancreas is rare low grade malignant tumor, especially in children, and that occurs chiefly in young women. This type of pancreatic tumor has more benign clinical features than any other type of the tumor of the pancreas dose, and long survival or even cure can be expected by aggressive treatment. We present a 10-year-old frmle patient with a papillary-cystic neoplasm of the pancreas, she had distant metastatic evidences to the liver, omentum and peritoneum. The tumor was totally excised with the spleen and greater omentum, and chemotherapy was carried out with vincristine and 5-FU. On the follow up studies, there are neither evidence of recurrence nor evidence of disease and the child has been doing well for two years.
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Acinar Cell Carcinoma of the Pancreas - A case report -
Soo Young Chung, Ik Yang, Yul Lee, Hai Jung Park, Hye Kyung Ahn
J Korean Cancer Assoc. 1995;27(4):692-696.
AbstractAbstract PDF
Acinar cell carcinoma in pancreas is uncommon and occurs in less than 2% among the epithelial non-endocrine pancreatic tumors. Acinar cell carcinoma is usually seen in the elderly patients and shows poor prognosis due to frequent metastasis. A 59 year old man was admitted because of indigestion and abdominal distension. A large, irregular, marginated, heterogeneous and hypodense mass with internal necrosis in pancreas was the characteristic CT feature of acinar cell carcinoma in pancreas. Celiac arteriogram showed a central hypovascular mass with sweeping of left gastric artery. Superic; mesenteric arteriography showed tortuous neovascularity along the peripheral portion of mass. Gross specimen showed an ill defined ovoid pink friable mass with a portion of necrotic yellow area. Light microscope showed gyriform or glendular pattern devided by fine fibrovascular stroma. The cells have round vesicular nuclei with prominent nucleoli located in relatively basally and abundant cytoplasm. Multiple electron-dense cytoplasmic zymogen granules in cytoplasm of the enlarged acinar cells on electron microscope can lead the diagnosis. We report the CT and angiographic findings of a case of acinar cell carcinoma in pancreas.
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Acinar Cell Carcinoma in a Pancreas : Report of a recurrent case
Eun Sook Lee, Ki Hoon Jung, Nam Ryul Kim, Seon Hahn Kim, Sae Min Kim, Chul Hwan Kim
J Korean Cancer Assoc. 1996;28(4):782-787.
AbstractAbstract PDF
Acinar cell carcinoma of the pancreas is a rare neoplasm arising from the exocrine cells of the pancreas, comprising no more than 1 to 2% of all pancreatic cancers. CT appearence of pancreatic acinar carcinoma has been described as a sharply circumscribed mass with central necrosis. We report a case of recurrent acinar cell carcinoma of the pancreas in a 41-year-old man who had distal pancreatectomy three years ago for primary acinar cell carcinoma.
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  • 19 Download
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