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

Sponsor
Triemli Hospital (Other)
Overall Status
Completed
CT.gov ID
NCT01286662
Collaborator
(none)
105
Enrollment
395
Duration (Months)

Study Details

Study Description

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.

Condition or Disease Intervention/Treatment Phase

    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 Design

    Study Type:
    Observational
    Actual Enrollment :
    105 participants
    Observational Model:
    Cohort
    Time Perspective:
    Retrospective
    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
    Study Start Date :
    Jan 1, 1978
    Actual Primary Completion Date :
    Dec 1, 2010
    Actual Study Completion Date :
    Dec 1, 2010

    Outcome Measures

    Primary Outcome Measures

    1. mortality [1978-2010 (as long as patients live)]

    Secondary Outcome Measures

    1. incidence of colorectal carcinoma [1978-2010 (entire patient life)]

    2. Incidence of desmoid tumors [1978-2010 (entire patient life)]

    Eligibility Criteria

    Criteria

    Ages Eligible for Study:
    N/A and Older
    Sexes Eligible for Study:
    All
    Accepts Healthy Volunteers:
    No
    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

    Contacts and Locations

    Locations

    No locations specified.

    Sponsors and Collaborators

    • Triemli Hospital

    Investigators

    • Principal Investigator: Matthias Turina, MD PhD, Triemli Hospital
    • Study Director: Hans P Simmen, MD, University of Zurich Hospital

    Study Documents (Full-Text)

    None provided.

    More Information

    Publications

    None provided.
    Responsible Party:
    , ,
    ClinicalTrials.gov Identifier:
    NCT01286662
    Other Study ID Numbers:
    • TriemliH
    First Posted:
    Jan 31, 2011
    Last Update Posted:
    Jan 31, 2011
    Last Verified:
    Jan 1, 2011
    Keywords provided by , ,
    gardner syndrome
    colorectal carcinoma
    desmoid tumor
    resection
    colon
    Additional relevant MeSH terms:
    Colorectal Neoplasms
    Fibromatosis, Aggressive
    Gardner Syndrome
    Syndrome

    Study Results

    No Results Posted as of Jan 31, 2011