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

Colorectal Carcinoma Clinical Trial

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01286662
• Other study ID #: TriemliH
• Status: Completed
• Phase: N/A
• First received: January 25, 2011
• Last updated: January 27, 2011
• Start date: January 1978
• Est. completion date: December 2010

Study information

• Verified date: January 2011
• Source: Triemli Hospital
• Contact: n/a
• Is FDA regulated: No
• Health authority: Switzerland: Laws and standards
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 105
• Est. completion date: December 2010
• Est. primary completion date: December 2010
• Accepts healthy volunteers: No
• Gender: Both
• Age group: N/A and older

Eligibility

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

Observational Model: Cohort, Time Perspective: Retrospective

Related Conditions & MeSH terms

• Adenomatous Polyposis Coli
• Colorectal Carcinoma
• Colorectal Neoplasms
• Desmoid Tumor
• Gardner Syndrome
• Syndrome

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
Triemli Hospital

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	mortality		1978-2010 (as long as patients live)	No
Secondary	incidence of colorectal carcinoma		1978-2010 (entire patient life)	No
Secondary	Incidence of desmoid tumors		1978-2010 (entire patient life)	No