RECRUITING

Genetic Analysis of Birt Hogg-Dube Syndrome and Characterization of Predisposition to Kidney Cancer

Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

Description

This study will investigate the genetic cause of Birt Hogg-Dube (BHD) syndrome and the relationship of this disorder to kidney cancer. BHD is a rare inherited condition characterized by papules, or bumps-benign tumors involving hair follicles-on the head and neck. People with BHD are at increased risk of developing kidney cancer. Scientists have identified the chromosome (strand of genetic material in the cell nucleus) that contains the BHD gene and the region of the gene on the chromosome. This study will try to learn more about: * The characteristics and type of kidney tumors associated with BHD * The risk of kidney cancer in people with BHD * Whether more than one gene causes BHD * The genetic mutations (changes) responsible for BHD Patients with known or suspected Birt Hogg-Dube syndrome, and their family members, may be eligible for this study. Candidates will be screened with a family history and review of medical records, including pathology reports for tumors, and films of computed tomography (CT) and magnetic resonance imaging (MRI) scans. Participants may undergo various tests and procedures, including the following: * Physical examination * Review of personal and family history with a cancer doctor, cancer nurses, kidney surgeon, and genetic counselor * Chest and other x-rays * Ultrasound (imaging study using sound waves) * MRI (imaging study using radiowaves and a magnetic field) * CT scans of the chest and abdomen (imaging studies using radiation) * Blood tests for blood chemistries and genetic testing * Skin evaluation, including a skin biopsy (surgical removal of a small skin tissue sample for microscopic evaluation) * Cheek swab or mouthwash to collect cells for genetic analysis * Lung function studies * Medical photography of skin lesions These tests will be done on an outpatient basis in either one day or over 3 to 4 days. When the studies are complete, participants will receive counseling about the findings and recommendations. Patients with kidney lesions may be asked to return periodically, such as every 3 to 36 months, based on their individual condition, to document the rate of progression of the lesions.

Official Title

Birt-Hogg-Dub(SqrRoot)(Copyright) Syndrome: Characterization of the FLCN Disease Gene and Predisposition to Renal Cancer, Cutaneous Fibrofolliculoma and Pulmonary Cysts

Quick Facts

Study Start:2002-05-13
Study Completion:N/A
Study Type:Not specified
Phase:Not Applicable
Enrollment:Not specified
Status:RECRUITING

Study ID

NCT00033137

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Ages Eligible for Study:2 Years
Sexes Eligible for Study:ALL
Accepts Healthy Volunteers:Yes
Standard Ages:CHILD, ADULT, OLDER_ADULT
Inclusion CriteriaExclusion Criteria
  1. 1. Individuals suspected or known to have phenotype or genotype suggestive of Birt-Hogg-Dube, such as:
  2. * Individuals with at least one histologically confirmed fibrofolliculomas; or
  3. * Individuals with clinical evidence of multiple skin papules (without fibrofolliculoma biopsy confirmation) and a personal or family history of spontaneous pneumothorax/or kidney cancer; or
  4. * Individuals with spontaneous pneumothorax and skin papules or kidney cancer and a positive family history of spontaneous pneumothorax, skin papules or kidney cancer; or
  5. * Individuals with a known germline FLCN gene mutation
  6. 2. Renal tumor histology consistent with BHD, including, but not limited to those suggestive of chromophobe, oncocytic neoplasm or oncocytoma.
  7. 3. All participants and guardians, for children younger than 18 years of age, must sign an informed consent document indicating their understanding of the investigational nature and the risks of this study before any protocol related studies are performed.
  8. 4. Participants must be greater than or equal to 2 years of age.
  9. 5. A relative (related by blood) of an individual with a confirmed or suspected diagnosis of BHD.
  1. Pregnancy or breastfeeding
  2. Severe psychiatric disorders
  3. Active substance abuse
  4. Unstable medical conditions
  5. Inability to comply with study requirements

Contacts and Locations

Study Contact

Deborah A Nielsen, R.N.
CONTACT
(240) 760-6247
deborah.nielsen@nih.gov
W. Marston Linehan, M.D.
CONTACT
(240) 858-3700
linehanm@mail.nih.gov

Principal Investigator

W. Marston Linehan, M.D.
PRINCIPAL_INVESTIGATOR
National Cancer Institute (NCI)

Study Locations (Sites)

National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
United States

Collaborators and Investigators

Sponsor: National Cancer Institute (NCI)

  • W. Marston Linehan, M.D., PRINCIPAL_INVESTIGATOR, National Cancer Institute (NCI)

Study Record Dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Registration Dates

Study Start Date2002-05-13
Study Completion DateN/A

Study Record Updates

Study Start Date2002-05-13
Study Completion DateN/A

Terms related to this study

Keywords Provided by Researchers

  • Pneumothorax
  • Kidney
  • Fibrofolliculoma
  • BHD
  • Neoplasms
  • Natural History

Additional Relevant MeSH Terms

  • Kidney Neoplasms
  • Kidney Cancer
  • Pneumothorax
  • FLCN Protein, Human