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Dermatologic Manifestations of Gardner Syndrome Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 08, 2016
 

Medical Care

Treatment of the cutaneous manifestations of Gardner syndrome depends on the symptomatic or cosmetic nature and the location of the cysts. Treatment is similar to that used for ordinary cysts and involves excision[21] or use of intralesional steroids if the cysts are inflamed.

Antiestrogens and anthracycline-containing regimens may have a favorable radiological response rate against desmoid tumors and may be a viable option in some patients.[22]

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Surgical Care

Colectomy[23] is recommended for Gardner syndrome patients if 30 or more polyps are detected on colonoscopy or if biopsy results reveal dysplasia or malignant degeneration. Preserving the rectum results in a 25-59% chance of rectal carcinoma occurring in Gardner syndrome patients; therefore, rectal mucosal resection is recommended. Total gastroduodenectomy with pancreatic preservation may be desirable in some settings.[24]

Generally, cutaneous findings do not require treatment. Osteomas may require excision if they are severely deforming or if they interfere with function.

Also see the General Surgery article Gardner Syndrome.

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Complications

In Gardner syndrome, polyps have a 100% risk of undergoing malignant transformation; therefore, surgical transection is indicated.

Females are at higher risk for thyroid carcinoma than males. Other neoplasms possible with Gardner syndrome include periampullary carcinoma, CNS tumors, osteosarcoma, chondrosarcoma, hepatoblastoma, and liposarcoma.

Desmoid tumors are a significant cause of the morbidity and mortality in those with Gardner syndrome; surgical procedures to excise them are complex.[8]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Sungnack Lee, MD Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea

Sungnack Lee, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Karen Allen, MD, and Raul Del Rosario, MD, to the development and writing of this article.

References
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  2. Ascari-Raccagni A, Baldari U, Righini MG. Cutaneous symptoms of Gardner's syndrome. J Eur Acad Dermatol Venereol. 1999 Jan. 12(1):80-1. [Medline].

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  12. Pena AH, Chaudhry A, Seidman RJ, Peyster R, Bangiyev L. Ectopic craniopharyngioma of the fourth ventricle in a patient with Gardner syndrome. Clin Imaging. 2016 Mar-Apr. 40 (2):232-6. [Medline].

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  14. Cazorla A, Viennet G, Uro-Coste E, Valmary-Degano S. Mucoepidermoid carcinoma: A yet unreported cancer associated with familial adenomatous polyposis. J Craniomaxillofac Surg. 2014 Apr. 42(3):262-4. [Medline].

  15. Kaplan I, Nicolaou Z, Hatuel D, Calderon S. Solitary central osteoma of the jaws: a diagnostic dilemma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008 Sep. 106(3):e22-9. [Medline].

  16. Pujol RM, Casanova JM, Egido R, Pujol J, de Moragas JM. Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome?. Pediatr Dermatol. 1995 Dec. 12(4):331-5. [Medline].

  17. Wachter-Giner T, Bieber I, Warmuth-Metz M, Brocker EB, Hamm H. Multiple pilomatricomas and gliomatosis cerebri--a new association?. Pediatr Dermatol. 2009 Jan-Feb. 26(1):75-8. [Medline].

  18. Van Epps KJ, Kuszyk BS, Hofmann LV, Fishman EK. Epidermoid inclusion cysts seen on CT of a patient with Gardner's syndrome. AJR Am J Roentgenol. 1999 Sep. 173(3):858-9. [Medline].

  19. Lanckohr C, Debiec-Rychter M, Müller O, Homann HH, Lehnhardt M, Herter P, et al. [Gardner fibroma: case report and discussion of a new soft tissue tumor entity]. Pathologe. 2010 Mar. 31(2):97-105. [Medline].

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