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

Descriptive Information
Brief Title † A Cohort Study of Patients Treated With Brachytherapy for Selected Desmoid Patients in Gardner Syndrome
Official Title † In Modern Era, Recurrent Desmoids Determine Outcome in Patients With Gardner Syndrome: A Cohort Study of Three Generations of an Adenomatous Polyposis Coli (APC-) Mutation-Positive Family Across 30 Years
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.
Detailed Description Since 1978, we have been following a family of 105 descendants with Gardner Syndrome (GS). Mutation carriers were screened by endoscopy, and colorectal resection was performed upon pending malignancy. Resectable desmoid tumors were excised, whereas large tumors of the abdominal wall were treated by a combination of brachytherapy (BT) and radiotherapy (RT). Outcome was analyzed with respect to length of tumor-free survival, and morbidity from surgery or radiotherapy. Results: 37 of 105 family members have GS. Preventive colorectal resections were performed in 16 patients (15%), with one death due to subsequent gastric cancer. In 4 patients who denied screening endoscopy, invasive tumors of the colon (3 patients) and stomach (one patient each) developed. Of 33 desmoid tumors, 10 (30%) were located in the mesentery, 17 (52%) in the abdominal wall, and 6 (18%) in extra-abdominal sites. Excision of 12 desmoids was performed in 8 patients (36%), and 4 were treated by a combination of BT and RT. Following BT/RT, all patients showed full or partial remission.
Study Phase N/A
Study Type † Observational
Study Design † Observational Model: Cohort, Time Perspective: Retrospective
Primary Outcome Measure † mortality
Secondary Outcome Measure † incidence of colorectal carcinoma
Condition † Gardner Syndrome Colorectal Carcinoma Desmoid Tumor
Intervention †
Study Arms / Comparison Groups
Publications *

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information
Recruitment Status †
Estimated Enrollment † 105
Start Date † January 1978
Completion Date December 2010
Primary Completion Date December 2010
Eligibility Criteria † Inclusion Criteria: - family with an identical adenomatous polyposis coli (APC-) germ line mutation Exclusion Criteria: - negative testing for adenomatous polyposis coli (APC-) germ line mutation
Gender Both
Ages N/A - N/A
Accepts Healthy Volunteers No
Contacts ††
Location Countries †
Administrative Information
NCT ID † NCT01286662
Organization ID TriemliH
Secondary IDs ††
Responsible Party
Study Sponsor † Triemli Hospital
Collaborators ††
Investigators † Principal Investigator: Matthias Turina, MD PhD, Triemli Hospital
Information Provided By
Verification Date January 2011
First Received Date † January 25, 2011
Last Updated Date January 27, 2011
† Required WHO trial registration data element.
†† WHO trial registration data element that is required only if it exists.
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