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

This study has been completed.
Sponsor:
Information provided by:
Triemli Hospital
ClinicalTrials.gov Identifier:
NCT01286662
First received: January 25, 2011
Last updated: January 27, 2011
Last verified: January 2011
  Purpose

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
Gardner Syndrome
Colorectal Carcinoma
Desmoid Tumor

Study Type: Observational
Study Design: 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

Resource links provided by NLM:


Further study details as provided by Triemli Hospital:

Primary Outcome Measures:
  • mortality [ Time Frame: 1978-2010 (as long as patients live) ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • incidence of colorectal carcinoma [ Time Frame: 1978-2010 (entire patient life) ] [ Designated as safety issue: No ]
  • Incidence of desmoid tumors [ Time Frame: 1978-2010 (entire patient life) ] [ Designated as safety issue: No ]

Enrollment: 105
Study Start Date: January 1978
Study Completion Date: December 2010
Primary Completion Date: December 2010 (Final data collection date for primary outcome measure)
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.

  Eligibility

Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population

Since 1978, we have been following a family of 105 descendants with Gardner syndrom. APC (adenomatous polyposis coli) mutation carriers were screened by endoscopy, and colorectal resection was performed upon pending malignancy. Desmoid tumors were treated by excision or radiotherapy and brachytherapy.

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
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01286662

Sponsors and Collaborators
Triemli Hospital
Investigators
Principal Investigator: Matthias Turina, MD PhD Triemli Hospital
Study Director: Hans P Simmen, MD University of Zurich Hospital
  More Information

No publications provided

Responsible Party: Matthias Turina, Triemli Hospital
ClinicalTrials.gov Identifier: NCT01286662     History of Changes
Other Study ID Numbers: TriemliH
Study First Received: January 25, 2011
Last Updated: January 27, 2011
Health Authority: Switzerland: Laws and standards

Keywords provided by Triemli Hospital:
gardner syndrome
colorectal carcinoma
desmoid tumor
resection
colon

Additional relevant MeSH terms:
Carcinoma
Colorectal Neoplasms
Gardner Syndrome
Adenomatous Polyposis Coli
Fibromatosis, Aggressive
Neoplasms, Glandular and Epithelial
Neoplasms by Histologic Type
Neoplasms
Intestinal Neoplasms
Gastrointestinal Neoplasms
Digestive System Neoplasms
Neoplasms by Site
Digestive System Diseases
Gastrointestinal Diseases
Colonic Diseases
Intestinal Diseases
Rectal Diseases
Adenomatous Polyps
Adenoma
Colonic Neoplasms
Neoplastic Syndromes, Hereditary
Intestinal Polyposis
Abnormalities, Multiple
Congenital Abnormalities
Genetic Diseases, Inborn
Fibroma
Neoplasms, Fibrous Tissue
Neoplasms, Connective Tissue
Neoplasms, Connective and Soft Tissue

ClinicalTrials.gov processed this record on July 24, 2014