Cohort of Well-differentiated Grade 3 Neuroendocrine Digestive Tumors

Study Overview
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Brief Summary
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The purpose of this study is to analyse clinical data of well-differentiated grade 3 digestive neuroendocrine tumors. These rare tumors may have a different disease evolution, response to chemotherapy and prognostic.

Detailed Description
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Digestive neuroendocrine tumors are rare tumors and cell differentiation is a major prognostic marker of neuroendocrine tumors. The 2010 WHO Classification defined three groups of tumor according to the combination of the morphological characteristics and the mitotic index and/or the Ki-67 index: Grade 1 and 2 corresponded to well differentiated neuroendocrine tumors whereas grade 3 corresponded to poorly differentiated lesions entitled neuroendocrine carcinomas (NEC). It was assumed that no well-differentiated neuroendocrine tumor with a mitotic- or a Ki-67- index above 20% existed. Recently, a proportion of neuroendocrine tumors corresponding to grade 3 neuroendocrine tumors with a proliferation- or Ki-67 index > 20% and with a well-differentiated morphology have been identified. This entity has been partially explored and may have a different survival than grade 3 NEC. Furthermore, targeted therapies, and used in pancreatic neuroendocrine tumors have not been assessed in this case. The TENpath network is a pathological network whose goal is the systematic reading of all diagnosed cases of neuroendocrine tumors. As part of this network, nearly 3.000 neuroendocrine tumors were reviewed by pathologist experts. Of all the reviewed tumors, 167 were identified as well-differentiated grade 3 neuroendocrine tumors, observed Ki-67(5.6%) confirming the existence of this entity. Treatment and follow-up of well-differentiated grade 3 tumors are not consensus-based and recommendations are exclusively based on experts' opinions. The purpose of this study is to define the characterization of this entity and evaluate the efficacy of chemotherapy on well-differentiated grade 3 digestive neuroendocrine tumors identified from the TENpath network.

Official Title
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Epidemiological Study of the Therapeutic Management of Well-differentiated Grade 3 (WHO Classification) Neuroendocrine Digestive Tumors From the Prospective Data of the French Neuroendocrine Tumors Registry (GTE)

Conditions
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Neuroendocrine Tumors

Intervention / Treatment
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* Drug: platinum chemotherapy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Well-differentiated grade 3 neuroendocrine digestive tumors Patient of 18 years old and more Exclusion Criteria: Patient opposed to data collection as part of the study Digestive neuroendocrine tumors Grade 1-2 Grade 3 poorly differentiated digestive neuroendocrine tumors Malignant disease diagnosed in the last 5 years (except basal cell of the skin and in situ cervical carcinoma) Other non-digestive neuroendocrine tumors Mixed neuroendocrine non neuroendocrine neoplasm

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| 1<br>Well-differentiated grade 3 gastrointestinal neuroendocrine tumors patients who receive first line platinum based chemotherapy | Drug: platinum chemotherapy<br>* First line platinum chemotherapy<br>|
| 2<br>Well-differentiated grade 3 gastrointestinal neuroendocrine tumors patients who receive receiving first line non-platinum chemotherapy | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Title : Overall survival | To compare the median of overall survival of Well-differentiated grade 3 gastrointestinal neuroendocrine tumors patients who receive first line platinum based chemotherapy versus patients receiving first line non-platinum chemotherapy | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Objective response rate |  | 12 months | 
| Progression Free Survival |  | 12 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Neuroendocrine Tumors, Epidemiology