A Cohort Study of Patients Treated With Brachytherapy for Selected Desmoid Patients in Gardner Syndrome

NCT01286662 | Completed

• 1 other identifier: TriemliH
• Study Type: observational
• Target: 105
• Locations: 0 countries
• Sites: N/A

Brief Summary

The purpose of this study is to assess the long-term outcome in a cohort of Gardner-Syndrome patients receiving prophylaxis and treatment for intestinal and non-intestinal tumors.

Conditions

Gardner Syndrome; Colorectal Carcinoma; Desmoid Tumor

Keywords

gardner syndrome; colorectal carcinoma; desmoid tumor; resection; colon

Outcome Measures

Primary Outcomes (1)

• mortality

  1978-2010 (as long as patients live)

Secondary Outcomes (2)

• incidence of colorectal carcinoma

  1978-2010 (entire patient life)

• Incidence of desmoid tumors

  1978-2010 (entire patient life)

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Since 1978, we have been following a family of 105 descendants with Gardner syndrom. APC (adenomatous polyposis coli) mutation carriers were screened by endoscopy, and colorectal resection was performed upon pending malignancy. Desmoid tumors were treated by excision or radiotherapy and brachytherapy.

You may qualify if:

• family with an identical adenomatous polyposis coli (APC-) germ line mutation

You may not qualify if:

• negative testing for adenomatous polyposis coli (APC-) germ line mutation

Sponsors & Collaborators

• Triemli Hospital: lead

Related Publications (1)

• Turina M, Pavlik CM, Heinimann K, Behrensmeier F, Simmen HP. Recurrent desmoids determine outcome in patients with Gardner syndrome: a cohort study of three generations of an APC mutation-positive family across 30 years. Int J Colorectal Dis. 2013 Jun;28(6):865-72. doi: 10.1007/s00384-012-1600-x. Epub 2012 Nov 1.

  PMID: 23114473 DERIVED

MeSH Terms

Conditions

Gardner Syndrome; Colorectal Neoplasms; Desmoid Tumors

Condition Hierarchy (Ancestors)

Adenomatous Polyposis Coli; Adenomatous Polyps; Adenoma; Neoplasms, Glandular and Epithelial; Neoplasms by Histologic Type; Neoplasms; Intestinal Neoplasms; Gastrointestinal Neoplasms; Digestive System Neoplasms; Neoplasms by Site; Neoplastic Syndromes, Hereditary; Digestive System Diseases; Gastrointestinal Diseases; Colonic Diseases; Intestinal Diseases; Intestinal Polyposis; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Rectal Diseases; Fibroma; Neoplasms, Fibrous Tissue; Neoplasms, Connective Tissue; Neoplasms, Connective and Soft Tissue

Study Officials

• Matthias Turina, MD PhD

  Triemli Hospital

  PRINCIPAL INVESTIGATOR

• Hans P Simmen, MD

  University of Zurich Hospital

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: January 25, 2011
• First Posted: January 31, 2011
• Study Start: January 1, 1978
• Primary Completion: December 1, 2010
• Study Completion: December 1, 2010
• Last Updated: January 31, 2011

Record last verified: 2011-01