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Study natural history of VHL pancreatic neuroendocrine tumors with yearly 68-Gallium DOTATATE PET/CT research scans.

Study natural history of VHL pancreatic neuroendocrine tumors without research scans.

68-Gallium DOTATATE, to be used in yearly PET/CT research scans

68-Gallium DOTATATE

Kristine J Villaruel

Naris Nilubol, M.D.

For more information at the NIH Clinical Center contact National Cancer Institute Referral Office

Background:

People with von Hippel-Lindau (VHL) can have problems with a variety of organs, such as the pancreas. The disease can cause tumors of the pancreas. This can result in life-threatening complications. Researchers want to learn more about these pancreatic tumors and how to better detect them. This may help them design better future treatment and care for people with VHL disease.

Objective:

To better understand VHL disease that affects the pancreas and to test whether adding a certain type of scan (68-Gallium DOTATATE PET/CT) can further detect tumors.

Eligibility:

People ages 12 and older with VHL that causes tumors and cysts to grow in the pancreas

Design:

Participants will be screened with their medical records and imaging studies.

Participants will have an initial evaluation:

Participants will have their body examined by different doctors. This will depend on what types of symptoms they have.

Participants will have blood and urine tests

Participants will have images made of their body using one or more machines: They made have a CT or PET/CT scan in which they lie on a table that moves through a big ring. They may have an MRI in which they lie on a table that moves into a big tube. They may have an ultrasound that uses a small stick that produces sound waves to look at the body.

After the first visit, participants will be asked to return to the NIH. Some of the tests performed at the first visit will be repeated. Depending on their disease status, visits will be once a year or every 2 years for life.

Background:

* Patients with the von Hippel-Lindau (VHL) familial cancer syndrome demonstrate manifestations in a variety of organs, including the pancreas. Pancreatic manifestations can range from benign cysts and micro cystic adenomas to neuroendocrine tumors of the pancreas which are capable of regional and distant metastases. These neuroendocrine tumors can result in life-threatening complications.
* This protocol is designed to identify VHL patients with pancreatic manifestations and to follow these patients with serial imaging studies, germ line genetic analysis, discovery of serum biomarkers, and novel imaging modalities such as 68-Gallium DOTATATE PET/CT scan.

Objective:

- To comprehensively and longitudinally evaluate the natural history of patients with VHL pancreatic neuroendocrine tumors and cystic lesions, estimating and defining their clinical spectrum.

Eligibility:

- Patients greater than or equal to 12 years of age diagnosed with VHL.

Design:

* Demographic data will be collected from the medical record and patient interview for each patient participant. Data will be securely stored in a computerized database.
* Patients will be evaluated by the urology, neurosurgery, brain oncology and/or ophthalmology personnel as indicated to rule out or manage other manifestations of VHL such as hemangioblastoma, renal cell cancer, and pheochromocytoma.
* Anatomical and functional clinical imaging studies, research blood and urine sample collection will be performed at each scheduled visit. Research 68-Gallium DOTATATE PET/CT imaging studies in adult subjects will be performed, no more than once per year.
* Surgical resection of solid lesions of the pancreas will be recommended based on previously published criteria.
* Based on our analysis of likelihood of tumor growth or risk of metastasis, data will be analyzed every two years and appropriate revisions will be made to the surgical management guidelines, if indicated by data analysis.
* Projected accrual will be 40 patients per year for a total of 15 years. Considering some patients may have a screen failure, we anticipate accruing of 740 patients on this protocol.

Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors

Evaluation of the Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors

.All IPD recorded in the medical record will be shared with intramural investigators upon request. @@@@@@In addition, all large scale genomic sequencing data will be shared with subscribers to dbGaP.

Characterization of the natural and clinical histories of VHL pancreatic neuroendocrine tumors and cystic lesions

mean uptake between these two groups of adult patients in which per patient uptake is defined as the average uptake of multiple lesions detected in each patient

median amount of time from initial presentation with pancreatic tumors to the time that surgery is recommended

National Cancer Institute (NCI)

Carcinoma

Carcinoma, Neuroendocrine

Carcinoid Tumor

Adenocarcinoma

NETest

Neuroendocrine tumors are derived from the neuroendocrine system of the gastroenteropancreatic and bronchopulmonary tract systems. Treatment options include surgery, medical and ablative therapies as well as peptide-receptor radionuclide therapy. Survival is linked to early and accurate diagnoses or to the effective detection of disease recurrence and/or treatment failure. One challenge is to develop accurate non-invasive blood tests that can detect neuroendocrine tumor activity. A second challenge is to evaluate the effectiveness of molecular biomarkers in the natural history of this disease. RegisterNET registry aims at collecting data and blood samples from patients presenting with a NET in the USA. Data will be entered prospectively and anonymized after informed consent. All physicians who treat neuroendocrine tumor patients are invited to participate to the registry. Data will be evaluated within regular time frames, focusing on diagnostic accuracy for biomarkers in the different types and grades of tumors, treatment modalities and patient outcomes (e.g. disease recurrence and survival), thereby contributing to an understanding of the role of biomarkers in tumor management.

Background: Survival for neuroendocrine tumors is linked to early and accurate diagnoses or to the effective detection of disease recurrence and/or treatment failure. Non-invasive biomarkers have been identified that can improve diagnosis and prognosis of patients. Little, however, is known about the utility of these markers in clinical practice.

Objective: To systematically and prospectively collect clinical information and blood samples from neuroendocrine tumors in the USA based a histologically confirmed diagnosis.

Methods: All neuroendocrine tumors (gastroenteropancreatic and pulmonary) are following informed consent. Data will be entered prospectively and anonymized. Patient history including a quality of life survey are completed by contributing physicians and blood sample is collected for analysis. All information will be transferred to the database. Evaluation of treatment modalities and patient outcomes (e.g. disease recurrence) will be assessed at follow-up times.

The primary objectives of the project are to:

* monitor patients with neuroendocrine tumors
* provide descriptive statistical analyses
* assessment of diagnostic accuracy of molecular-based blood tests

The secondary objectives of the project include:

* assessment of disease recurrence
* analysis of patient survival

Analyses will include:

1. Descriptive statistical analyses including demographics, site, treatment, QoL assessment.
2. Clinical follow-up and blood chemistry results.
3. Correlation analyses between blood results and clinical data. This will include assessment of the time at which the blood chemistry results significantly (and consistently) increases and the time of tumor recurrence and an evaluation whether the change in blood results is predictive of disease recurrence.

Blood collection (RNA stabilization tube) used for neuroendocrine tumor biomarker measurements

RegisterNET - A Registry for Neuroendocrine Tumors in the USA

A Registry for Neuroendocrine Tumors in the USA

Image-based identification of recurrent tumor disease

Histological confirmation of neuroendocrine tumor

Questionnaire to evaluate quality of life status

Biomarker prediction of treatment response and disease relapse

Wren Laboratories LLC

Respiratory Tract (Lung and Bronchial) Diseases

Lung Neoplasms

Gastro-enteropancreatic Neuroendocrine Tumor

Participants with histologically or cytologically proven diagnosis of any grade, any stage NET of gastroenteropancreatic (GEP) or lung origin; In the first stage of the study (initial 50 patients) only potential participants with stage IV, well-differentiated tumors (G1/G2) will be enrolled.

Participants with histologically or cytologically proven diagnosis of any grade, any stage gastrointestinal (GI) malignancies.

5 mL of blood will be drawn from participants for testing.

Jonathan Strosberg, M.D.

The purpose of this study is to evaluate how well an investigational blood test performs. The study will look at the sensitivity and specificity of a blood-based multitranscriptome assay (NETest).

Clinical Utility Assay as a Biomarker for Gastroenteropancreatic and Lung Neuroendocrine Tumors

The Clinical Utility of a Blood-Based Multitranscriptome Assay as a Biomarker for Gastroenteropancreatic and Lung Neuroendocrine Tumors

Investigators have designed the study to test the null hypothesis that the NETest has a sensitivity and specificity of 70% or less in NET patients. The sample size calculation has been based on the assumption that a sensitivity and specificity of greater than 90% would generate further interest in the test for unselected NET patients. Power and type 1 error will be 99% and 5% respectively. Under this model, 80 or more positive tests in the cohort of 100 patients with NETs would lead to the rejection of the null hypothesis, suggesting that the NETest is sensitive. Likewise, 80 or more negative tests in 100 patients without NETs will suggest that NETest is specific. An interim analysis will be performed to rule out the futility of the NETest. Futility will be defined as a rate of false positive or false negative \>25%. Hence, if 12 or more false positives or negatives are observed among the first 50 participants, the study will be suspended, pending review by the investigators.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors	NCT04074135	VHL Pancreatic ... Von Hippel-Lind... Neuroendocrine ...	68-Gallium DOTA...	12 Years -	National Institutes of Health Clinical Center (CC)
RegisterNET - A Registry for Neuroendocrine Tumors in the USA	NCT02270567	Neuroendocrine ... Carcinoid Neuroendocrine ...	NETest	21 Years -	Wren Laboratories LLC
Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors	NCT04074135	VHL Pancreatic ... Von Hippel-Lind... Neuroendocrine ...	68-Gallium DOTA...	12 Years -	National Institutes of Health Clinical Center (CC)
RegisterNET - A Registry for Neuroendocrine Tumors in the USA	NCT02270567	Neuroendocrine ... Carcinoid Neuroendocrine ...	NETest	21 Years -	Wren Laboratories LLC
Clinical Utility Assay as a Biomarker for Gastroenteropancreatic and Lung Neuroendocrine Tumors	NCT02948946	Gastroenteropan... Lung Neuroendoc...	NETest	18 Years -	H. Lee Moffitt Cancer Center and Research Institute
RegisterNET - A Registry for Neuroendocrine Tumors in the USA	NCT02270567	Neuroendocrine ... Carcinoid Neuroendocrine ...	NETest	21 Years -	Wren Laboratories LLC

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Spots Global Cancer Trial Database for netest

Every month we try and update this database with for netest cancer trials from around the world to help patients and their families find trials that might be right for them. We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Every month we try and update this database with for netest cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.