Tissue Procurement and Natural History Study of Neuroendocrine Neoplasms (NENs) Including Adrenocortical Carcinoma (ACC)

NCT05237934 | Recruiting

• 2 other identifiers: 10000491000491-C
• Study Type: observational
• Target: 300
• Locations: 1 country
• Sites: 1

Brief Summary

Background: Neuroendocrine neoplasm (NENs)are rare cancers arising from the neuroendocrine cells and can affect almost any part of the body. They vary from low grade neuroendocrine tumors (NETs) to high grade neuroendocrine carcinomas (NECs). These tumors often occur in the gastrointestinal tract, pancreas, lungs, adrenal medulla (pheochromocytomas) or adrenal cortex (adrenocortical cancer) and other areas of the body mentioned below:

• Gastroenteropancreatic neuroendocrine tumors (GEP-NET): stomach, duodenum, pancreas, colon, appendix, etc.
• Liver and gallbladder
• Adrenal tumors
• Pituitary gland
• Thyroid gland: medullary thyroid carcinoma
• Parathyroid tumors
• Pulmonary neuroendocrine tumors: typical and atypical carcinoid, small cell lung cancer (SCLC), large cell neuroendocrine carcinoma (LCNEC)
• Extrapulmonary small cell cancer
• Peripheral nervous system tumors: paraganglioma, neuroblastoma)
• Breast and genitourinary tract Their rates are rising in the United States and worldwide. Researchers want to learn more about NENs through this natural history study. Objective: To study the natural history of people with NENs and obtain samples from them to learn more about the disease. The clinical management of all NETs is not standardized, with only a few FDA-approved therapies and we would like to learn which combination therapeutic approach should be used, how long treatment should be continued, and in what subgroup of NENs a particular treatment option should be used. Eligibility: People aged 18 and older who have or are suspected to have NENs or ACC. Design: Participants will be screened with a medical history. Participants will have a physical exam. Their symptoms and their ability to perform their normal activities will be reviewed. They will have blood and urine tests. Participants will receive recommendations for managing their disease and potential treatment options. They will be able to ask as many questions as they would like. Participants may provide saliva and blood, a for research. They will give tumor samples from a previous surgery or biopsy. Participants may have optional biopsies. During biopsies, cancer tissue will be obtained using a needle and syringe. Tissue will be taken from the liver, lung, or a lymph node. Participants may have an imaging scan or ultrasound to help locate the tumor or area to be biopsied. They will receive local anesthesia and may be sedated. Participants will complete a questionnaire about their family medical history. Participants will have follow-up visits every 12 months. They will have physical exams and give samples. If their health changes, they may have extra visits. If they cannot visit NIH, they (or their doctor) will be contacted by phone or email. Participants will take part in the study for all their life.

Conditions

Neuroendocrine Tumors; Carcinoma, Neuroendocrine

Keywords

Clinical Outcome; Advice in the Management of Cancer; Patterns of Disease Progression; Response or Lack of Response to Therapeutic Interventions; Natural History

Outcome Measures

Primary Outcomes (1)

• natural history of neuroendocrine neoplasms including ACC

  To comprehensively and longitudinally evaluate the natural history of participants with NENs and allow sample acquisition for use in the study of NENs

  ongoing

Study Arms (1)

Cohort 1

Participants with confirmed/suspected NENs including ACC

Eligibility Criteria

• Age: 18 Years - 120 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

primary clinical

You may qualify if:

• Age \>= 18 years old
• The ability of the participant to understand and the willingness to sign a written consent document.
• Participants with the documentation of:
• histological or cytological confirmation of NENs or adrenocortical cancer
• biochemical evidence of neuroendocrine tumor (serum/urinary) based on elevated levels of chromogranin A, pancreatic polypeptide, neuron-specific enolase, vasoactive intestinal polypeptide, serotonin (urinary 5-hydroxyindoleacetic acid (5-HIAA)), gastrin, somatostatin, catecholamines, metanephrines, calcitonin, fasting insulin, Cpeptide (proinsulin), glucagon, anterior pituitary hormones
• Suspicion of NEN (from any site/origin) on axial imaging (computed tomography (CT)/ magnetic resonance imaging (MRI) / fluorodeoxyglucose (FDG) positron emission tomography (PET) / 68Ga-Dotatate scan
• a germline genetic variant that predisposes to NETs including ACC.

You may not qualify if:

• None

Sponsors & Collaborators

• National Cancer Institute (NCI): lead

Study Sites (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

RECRUITING

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Neuroendocrine Tumors; Carcinoma, Neuroendocrine

Condition Hierarchy (Ancestors)

Neuroectodermal Tumors; Neoplasms, Germ Cell and Embryonal; Neoplasms by Histologic Type; Neoplasms; Neoplasms, Nerve Tissue; Adenocarcinoma; Carcinoma; Neoplasms, Glandular and Epithelial

Study Officials

• Jaydira Del Rivero, M.D.

  National Cancer Institute (NCI)

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Anna Liza F Rivero

CONTACT

(240) 858-7946; anna.rivero@nih.gov

Jaydira Del Rivero, M.D.

CONTACT

(240) 858-3851; delriveroj@mail.nih.gov

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 5, 2022
• First Posted: February 14, 2022
• Study Start: April 25, 2022
• Primary Completion (Estimated): December 31, 2032
• Study Completion (Estimated): December 31, 2032
• Last Updated: November 12, 2025

Record last verified: 2025-11-06

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, SAP, ICF
• Time Frame: Clinical data available during the study and indefinitely.@@@@@@Genomic data will be available once genomic data are uploaded per protocol GDS plan for as long as database is active.
• Access Criteria: Clinical data will be made available via subscription to BTRIS and with the permission of the study PI.@@@@@@Genomic data will be made available via dbGaP through requests to the data custodians.

Locations

US (1)