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

Completed

• Conditions: Gardner Syndrome; Colorectal Carcinoma; Desmoid Tumor

• Registration Number: NCT01286662
• Lead Sponsor: Triemli Hospital

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 Timeline

• Study Start: 1978-01
• Primary Completion: 2010-12
• Study Completion: 2010-12
• Status Verified: 2011-01
• Study First Submitted: 2011-01-25
• Study First Submitted QC: 2011-01-27
• Last Update Submitted: 2011-01-27
• Study First Posted: 2011-01-31
• Last Update Posted: 2011-01-31

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 105

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
mortality	1978-2010 (as long as patients live)

Secondary Outcome Measures

Name	Time	Method
incidence of colorectal carcinoma	1978-2010 (entire patient life)
Incidence of desmoid tumors	1978-2010 (entire patient life)