scholarly journals Comparison of genetic alterations in neuroendocrine tumors: frequent loss of chromosome 18 in ileal carcinoid tumors

2005 ◽  
Vol 18 (8) ◽  
pp. 1079-1087 ◽  
Author(s):  
Gordon G Wang ◽  
James C Yao ◽  
Samidha Worah ◽  
Jill A White ◽  
Rene Luna ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Genetic Alterations ◽  
Carcinoid Tumors ◽  
Chromosome 18 ◽  
Ileal Carcinoid

Common pathogenetic mechanism involving human chromosome 18 in familial and sporadic ileal carcinoid tumors

2010 ◽  
Vol 50 (2) ◽  
pp. 82-94 ◽  
Author(s):  
Janet L. Cunningham ◽  
Teresita Díaz de Ståhl ◽  
Tobias Sjöblom ◽  
Gunnar Westin ◽  
Jan P. Dumanski ◽  
...  
Keyword(s):  
Human Chromosome ◽  
Carcinoid Tumors ◽  
Pathogenetic Mechanism ◽  
Chromosome 18 ◽  
Ileal Carcinoid

scholarly journals Molecular Pathology of Well-Differentiated Pulmonary and Thymic Neuroendocrine Tumors: What Do Pathologists Need to Know?

2021 ◽  
Vol 32 (1) ◽  
pp. 154-168 ◽  
Author(s):  
Marco Volante ◽  
Ozgur Mete ◽  
Giuseppe Pelosi ◽  
Anja C. Roden ◽  
Ernst Jan M. Speel ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Young Patients ◽  
Carcinoid Tumors ◽  
Neuroendocrine Neoplasms ◽  
Grey Zone ◽  
Cell Hyperplasia ◽  
Ki 67 ◽  
Familial Form ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

AbstractThoracic (pulmonary and thymic) neuroendocrine tumors are well-differentiated epithelial neuroendocrine neoplasms that are classified into typical and atypical carcinoid tumors based on mitotic index cut offs and presence or absence of necrosis. This classification scheme is of great prognostic value but designed for surgical specimens, only. Deep molecular characterization of thoracic neuroendocrine tumors highlighted their difference with neuroendocrine carcinomas. Neuroendocrine tumors of the lung are characterized by a low mutational burden, and a high prevalence of mutations in chromatin remodeling and histone modification-related genes, whereas mutations in genes frequently altered in neuroendocrine carcinomas are rare. Molecular profiling divided thymic neuroendocrine tumors into three clusters with distinct clinical outcomes and characterized by a different average of copy number instability. Moreover, integrated histopathological, molecular and clinical evidence supports the existence of a grey zone category between neuroendocrine tumors (carcinoid tumors) and neuroendocrine carcinomas. Indeed, cases with well differentiated morphology but mitotic/Ki-67 indexes close to neuroendocrine carcinomas have been increasingly recognized. These are characterized by specific molecular profiles and have an aggressive clinical behavior. Finally, thoracic neuroendocrine tumors may arise in the background of genetic susceptibility, being MEN1 syndrome the well-defined familial form. However, pathologists should be aware of rarer germline variants that are associated with the concurrence of neuroendocrine tumors of the lung or their precursors (such as DIPNECH) with other neoplasms, including but not limited to breast carcinomas. Therefore, genetic counseling for all young patients with thoracic neuroendocrine neoplasia and/or any patient with pathological evidence of neuroendocrine cell hyperplasia-to-neoplasia progression sequence or multifocal disease should be considered.

Protocol for the Examination of Specimens From Patients With Neuroendocrine Tumors (Carcinoid Tumors) of the Appendix

2010 ◽  
Vol 134 (2) ◽  
pp. 171-175 ◽  
Author(s):  
Mary Kay Washington ◽  
Laura H. Tang ◽  
Jordan Berlin ◽  
Philip A. Branton ◽  
Lawrence J. Burgart ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Carcinoid Tumors

Use of Molecular Profiling to Guide Treatment Decisions in Patients with Neuroendocrine Tumors: Preliminary Results

2016 ◽  
Vol 82 (4) ◽  
pp. 369-375 ◽  
Author(s):  
Holt S. Cutler ◽  
Paul Ogando ◽  
Joshua H. Uhr ◽  
Dani O. Gonzalez ◽  
Richard R.P. Warner ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Clinical Symptoms ◽  
Case Series ◽  
Molecular Profiling ◽  
Serum Markers ◽  
Carcinoid Tumors ◽  
Molecular Profile ◽  
Chemotherapy Response ◽  
Choice Of Treatment ◽  
Selection Of

This case series demonstrates the potential of molecular profiling to improve selection of anti-tumor therapies in the treatment of patients with neuroendocrine and carcinoid tumors. Carcinoid tumors resected at one institution over a 3-year period were sent for molecular profiling to guide choice of treatment. Potentially beneficial therapies were identified based on the measured expression of 20 proteins and oncogenes and a comprehensive review of the chemotherapy response literature. The clinical charts of 41 patients were reviewed retrospectively, and 12 were selected as representatives of the range of effects molecular profiling has on carcinoid treatment. Their presentation, molecular profile results, treatment, and disease progression is reviewed in the following case series. A total of nine patients were treated with drugs identified as potentially beneficial by molecular profile reports. These include capecitabine, 5-fluorouracil, temozolomide, oxaliplatin, and gemcitabine. Based on clinical symptoms, serum markers of disease, and radio-graphic evidence five of nine patients responded to treatment, two had mixed responses, and two did not respond to treatment. At this early juncture, our critique of molecular profiling for neuroendocrine tumors is favorable, as a significant number of our patients responded to drugs identified by molecular profiling as potentially beneficial.

scholarly journals Jejuno-Ileal Carcinoid Tumors Are Rarely Amenable to Endoscopic Resection

2011 ◽  
Vol 140 (5) ◽  
pp. S-874
Author(s):  
Joshua E. Melson ◽  
Raja Koteswar Dhanekula ◽  
Peter Eichenseer ◽  
Abhitabh Patil ◽  
Kateri A. Evans ◽  
...  
Keyword(s):  
Endoscopic Resection ◽  
Carcinoid Tumors ◽  
Ileal Carcinoid

Phase II study of continuous infusion fluorouracil and interferon alfa-2b in the palliation of malignant neuroendocrine tumors.

1995 ◽  
Vol 13 (6) ◽  
pp. 1486-1492 ◽  
Author(s):  
H J Andreyev ◽  
P Scott-Mackie ◽  
D Cunningham ◽  
V Nicolson ◽  
A R Norman ◽  
...  
Keyword(s):  
Continuous Infusion ◽  
Neuroendocrine Tumors ◽  
Phase Ii ◽  
Advanced Disease ◽  
Phase Ii Study ◽  
Symptom Control ◽  
Objective Response ◽  
Interferon Alfa ◽  
Carcinoid Tumors ◽  
Disease Stabilization

PURPOSE AND METHODS Twenty-four patients with rapidly progressive neuroendocrine tumors were treated with a new regimen of continuous infusion fluorouracil for 20 weeks (200 mg/m2/d) together with interferon alfa-2b (5 MU three times per week). Maintenance interferon alfa at the same dose was continued after the initial 20-week period. RESULTS Of 15 patients with carcinoid tumors, seven (47%) had an objective response, with a median duration of 20.5 months (range, 8.5 to 41), and five (33%) had stabilization of disease for between 3.5 and 42 months. Improvement in symptoms was reported by 10 patients (67%). Three early deaths occurred, all in patients with advanced disease. Of nine patients with neuroendocrine tumors other than carcinoid, three (33%) had an objective response that lasted 2.5 to 24.5 months, and five had disease stabilization for between 2.5 and 16 months. CONCLUSION These data, particularly in respect to carcinoid tumors, are encouraging, especially since serious complications from treatment were limited. This regimen is not generally toxic, is well tolerated, and offers useful palliation and symptom control in patients with disease that does not respond to simple pharmacologic manipulations.

Recurrence rates of resected appendix carcinoid tumors: A 20-year experience at Memorial Sloan Kettering Cancer Center.

2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 221-221
Author(s):  
Jennifer Murphy ◽  
Andrew Rosowicz ◽  
Leonard Saltz ◽  
Philip Paty ◽  
Michael P. La Quaglia ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Lymph Node Involvement ◽  
Right Hemicolectomy ◽  
Carcinoid Tumors ◽  
Cancer Center ◽  
Single Institution ◽  
Recurrence Rates ◽  
Incidental Appendectomy ◽  
Average Size

221 Background: NETs of the appendix are rare neoplasms most often discovered during incidental appendectomy. Risk of nodal metastases based on single institution series has suggested that appendix carcinoid tumors > 2 cm should undergo a right colectomy. Unlike other tumors, lymph node involvement does not change management given the lack of adjuvant therapies. We sought to evaluate our experience of patients with resected appendix carcinoid, the risk of recurrence, and the benefit of surveillance following resection. Methods: A single institution database of all patients with diagnosis of neuroendocrine tumors was queried for all resected appendix carcinoid tumors. Excluded were patients with large and small cell neuroendocrine tumors, goblet carcinoid, and patients with inaccessible patient records. Results: 88 patients were included from August, 1994 through December, 2013. The mean age at diagnosis was 37.1(9.9-75.8). There were 19 (22%) children (less than 18 years of age) and 69 (78%) adults. Females represented 72% of all patients. All patients had well differentiated tumors. Median follow-up was 2.5 years (1-16.5 years, 45% of which had longer than 3-year follow-up). 43/88 (49%) patients underwent right hemicolectomy (average size 2 cm) and 45/88 (51%) simple appendectomy (average size 0.9 cm). Most patients were followed with CT scans and laboratory evaluations and frequency of surveillance was inconsistent. There were no recurrences (0/88). Ten-year survival was 100%. Conclusions: This is one of the largest series of resected appendix carcinoid reported and no patients recurred. Prognosis was excellent and in the absence of metastatic disease, excessive imaging and/or laboratory testing should be avoided. We question the use of routine right hemocolectomy in these patients.

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