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

NCT ID: NCT01286662

Last Updated: 2011-01-31

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

105 participants

Study Classification

OBSERVATIONAL

Study Start Date

1978-01-31

Study Completion Date

2010-12-31

Brief Summary

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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

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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.

Conditions

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Gardner Syndrome Colorectal Carcinoma Desmoid Tumor

Keywords

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gardner syndrome colorectal carcinoma desmoid tumor resection colon

Study Design

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Observational Model Type

COHORT

Study Time Perspective

RETROSPECTIVE

Eligibility Criteria

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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
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Triemli Hospital

OTHER

Sponsor Role lead

Responsible Party

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Triemli Hospital

Principal Investigators

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Matthias Turina, MD PhD

Role: PRINCIPAL_INVESTIGATOR

Triemli Hospital

Hans P Simmen, MD

Role: STUDY_DIRECTOR

University of Zurich Hospital

References

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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.

Reference Type DERIVED
PMID: 23114473 (View on PubMed)

Other Identifiers

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TriemliH

Identifier Type: -

Identifier Source: org_study_id