scholarly journals Genomic characterization of a well-differentiated grade 3 pancreatic neuroendocrine tumor

2019 ◽  
Vol 5 (3) ◽  
pp. a003814 ◽  
Author(s):  
Laura M. Williamson ◽  
Michael Steel ◽  
Jasleen K. Grewal ◽  
My Lihn Thibodeau ◽  
Eric Y. Zhao ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Pancreatic Neuroendocrine Tumor ◽  
Genomic Characterization ◽  
Well Differentiated ◽  
Grade 3

scholarly journals Case Report: Grade 2 Metastatic Pancreatic Neuroendocrine Tumor With Progression of One Metastasis After Pregnancy to Grade 3 Large-Cell Neuroendocrine Carcinoma: One Case Cured by Resection With Genomic Characterization of the Two Components

2021 ◽  
Vol 11 ◽  
Author(s):  
Jean-Luc Raoul ◽  
Marie-Françoise Heymann ◽  
Frédéric Dumont ◽  
Alain Morel ◽  
Hélène Senellart ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Pancreatic Neuroendocrine Tumor ◽  
Large Cell ◽  
Genomic Signatures ◽  
Pancreatic Net ◽  
Genomic Characterization ◽  
Poorly Differentiated ◽  
Grade 3

Temporal and spatial tumor heterogeneity can be observed in pancreatic neuroendocrine tumor. We report the case of a young woman with long term stabilization of a G2 metastatic pancreatic NET that, after pregnancy, suddenly progressed into one single liver metastasis corresponding to a transformation into G3 large-cell neuroendocrine cancer. The patient underwent liver resection (the progressive and one dormant metastasis). With a 45 months follow-up the patient is without evolutive disease. Exome sequencing of the two metastases revealed completely different genomic signatures and gene alterations: the dormant metastasis was MSS without any gene alteration; the poorly differentiated tumor was MSI, with gain of many mutations including MEN1, BCL2, MLH1 and TP53 corresponding to a mutational signature 11. Could temozolomide play a role in this transformation?

scholarly journals Current Paradigm of Treatment for Pancreatic Neuroendocrine Tumor

2021 ◽  
Vol 26 (1) ◽  
pp. 24-32
Author(s):  
Min Je Sung ◽  
Moon Jae Chung
Keyword(s):  
Cell Proliferation ◽  
Medical Treatment ◽  
Neuroendocrine Tumor ◽  
Standard Treatment ◽  
Pancreatic Neuroendocrine Tumor ◽  
Somatostatin Analogues ◽  
Proliferation Index ◽  
Ki 67 ◽  
Grade 3 ◽  
Cell Proliferation Index

Pancreatic neuroendocrine tumor (PNET) refer to tumors originating from the islet of Langerhans and shows various prognosis based on the presence or absence of symptoms due to hormone secretion, the Ki-67 cell proliferation index, and the histologic grade, and according to the degree of disease progression defined by the tumor-node-metastasis (TNM) stage classification. The purpose of medical treatment for PNET is to control symptoms or inhibit tumor growth. Somatostatin analogues can be administered for the purpose of controlling symptoms caused by the secretion of specific hormones, and are accepted as effective drugs for inhibiting the progression of G1/G2 tumors based on World Health Organization (WHO) classification with a Ki-67 cell proliferation index less than 20%. Among the molecularly targeted agents, everolimus and sunitinib can be considered in patients with WHO G1/G2 PNET showing progression after somatostatin analog therapy. Cytotoxic chemotherapy is generally administered to patients with large tumor volume and rapidly progressing metastatic NET, and etoposide/cisplatin combination therapy has been considered as a standard treatment. For the patient group of Grade 3 PNET (well differentiated) newly classified by the WHO 2017 classification, guidelines for standard treatment have not yet been established. As it has been reported, studies are needed to evaluate the treatment response rate of somatostatin analogues or molecularly targeted therapies for the patient with Grade 3 PNET. It is important to consider a multidisciplinary approach with all possible treatment options including medical treatment, radical resection of primary or metastatic lesions, liver-directed therapies, and peptide receptor radionuclide therapy for the patients with PNET.

Establishment of the First Well-differentiated Human Pancreatic Neuroendocrine Tumor Model

2018 ◽  
Vol 16 (3) ◽  
pp. 496-507 ◽  
Author(s):  
Daniel Benten ◽  
Yasmin Behrang ◽  
Ludmilla Unrau ◽  
Victoria Weissmann ◽  
Gerrit Wolters-Eisfeld ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Tumor Model ◽  
Pancreatic Neuroendocrine Tumor ◽  
Well Differentiated

Giant Primary Neuroendocrine Neoplasms of the Liver: Report of 2 Cases With Molecular Characterization

2019 ◽  
Vol 27 (8) ◽  
pp. 893-899
Author(s):  
Laura G. Pastrián ◽  
Ignacio Ruz-Caracuel ◽  
Raul S. Gonzalez
Keyword(s):  
World Health Organization ◽  
Neuroendocrine Tumor ◽  
Large Cell ◽  
The Other ◽  
World Health ◽  
Neuroendocrine Neoplasms ◽  
Molecular Testing ◽  
Well Differentiated ◽  
Grade 3 ◽  
Health Organization

Primary neuroendocrine neoplasms of the liver have occasionally been reported in the liver, though many reports do not convincingly exclude metastases. In this article, we report 2 “giant” hepatic neuroendocrine lesions without evidence of a primary elsewhere after clinical workup. One occurred in a 21-year-old male; the lesion was a large cell neuroendocrine carcinoma measuring 24 cm. The patient died of disease in 10 months. The other occurred in a 25-year-old patient, was 18 cm wide, and was diagnosed as a well-differentiated neuroendocrine tumor, World Health Organization grade 3. The patient died of disease after 30 months. Molecular testing demonstrated only the presence of TP53 mutations in common. These cases expand our knowledge of seemingly primary neuroendocrine neoplasms of the liver, in particular, giant cases measuring more than 8 cm. Guidelines for clinical workup and therapy for these lesions remain unclear, but future thorough workup of such cases is necessary for specific characterization.

Genomic characterization of grade 3 endometrial carcinoma

2014 ◽  
Vol 133 ◽  
pp. 134-135
Author(s):  
J.G. Cohen ◽  
M.T. Goodman ◽  
B.Y. Karlan ◽  
C. Walsh
Keyword(s):  
Endometrial Carcinoma ◽  
Genomic Characterization ◽  
Grade 3

Characterization of signaling pathways and molecular mechanisms underlying kisspeptin response in pancreatic neuroendocrine tumor (panNETs) cells

2021 ◽  
Author(s):  
C Fuentes-Fayos Antonio ◽  
Alors-Pérez Emilia ◽  
Pedraza Arévalo Sergio ◽  
D. Herrera-Martínez Aura ◽  
Angel Días-Pérez Jose ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Signaling Pathways ◽  
Molecular Mechanisms ◽  
Pancreatic Neuroendocrine Tumor

Gastroenteropancreatic neuroendocrine tumors: clinicopathological evaluation at Shifa International Hospital, Islamabad. A single centre experience

2020 ◽  
pp. 1-14
Author(s):  
Nadira Mamoon ◽  
Hania Naveed ◽  
Mariam Abid ◽  
Humaira Nasir ◽  
Imran Nazir Ahmad ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Neuroendocrine Carcinoma ◽  
World Health ◽  
Gastroenteropancreatic Neuroendocrine Tumors ◽  
Single Centre Experience ◽  
Who 2010 Classification ◽  
Well Differentiated ◽  
Grade 3 ◽  
Health Organization

Abstract Objective: Clinicopathological features of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) have rarely been studied in the Pakistani population. We investigated the clinical characteristics of these tumors according to the updated World Health Organization (WHO) 2010 classification. Methods: The data of Shifa International Hospital, Islamabad was retrospectively analysed for pathologically confirmed GEP-NETs from January 2013 to March 2018. Results: One hundred and eighteen patients (mean age, 52.2 years; male, 55.1%) were identified. 83.1% of the patients were symptomatic including5.1% functional tumors. Pancreas (28%) was the most frequent primary site noted. The most common histologic type was well differentiated neuroendocrine tumor (WDNET) in 81.4% followed by neuroendocrine carcinoma (NEC) in 16.1%. 45.8% cases of WDNET were grade 1, 27.1% were grade 2, and 8.5% were grade 3.15.3% had distant metastasis at the time of diagnosis with liver (77.7%) as the most common metastatic site. Synaptophysin positivity was seen in 96.8% of grade 1 & grade 2 WDNET, 100% of grade 3 WDNET and 92.3% of NEC and chromogranin was positive in 94.2% of grade 1 &grade 2 WDNET, 83.3% of grade 3 WDNET and 45.4% of NEC. Conclusion: GEP-NETs showed a wide clinicopathological spectrum. Pancreas is the most site of involvement by the GEP-NET however grade 3 WDNET had a predilection for the colon. Small cell carcinomas were commonly observed in esophagus. Keywords: Gastroenteropancreatic neuroendocrine tu­mor, well differentiated neuroendocrine tumor, neuroendocrine carcinoma. Continuous...

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