Ann Dermatol.  2010 Nov;22(4):418-421. 10.5021/ad.2010.22.4.418.

A Case of Gardner's Syndrome Associated with Desmoid Tumor

• Chung J
• Namkoong S
• Jung KE
• Park JW
• Park BC
• Cinn YW
• Kim MH

• KMID: 2266178
• DOI: http://doi.org/10.5021/ad.2010.22.4.418

Abstract

Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner's syndrome. Herein, we report a 16-year-old woman with Gardner's syndrome complicated by desmoid tumors on the right subscapular area.

Keyword

Desmoid tumor; Familial polyposis coli; Gardner's syndrome

MeSH Terms

Adenomatous Polyposis Coli
Adolescent
Female
Fibroblasts
Fibromatosis, Aggressive
Gardner Syndrome
Humans

Figure

• 

  Fig. 1 Skin colored elevated tumor on right subscapular area, measuring 7×4 cm.

• 

  Fig. 2 Fibrous dysplasia involving facial and cranial bones.

• 

  Fig. 3 Endoscopic findings. Numerous small polyps are observed in stomach (A) and colon (B).

• 

  Fig. 4 Histopathology of right subscapular tumor. (A) Dense collagen bundles in dermis (H&E, ×40). (B) Thickened collagen bundles with interspersed spindle cells and fibroblast (H&E, ×200).

Reference

1. Smith WG. Multiple polyposis, Gardner's syndrome and desmoid tumors. Dis Colon Rectum. 1958. 1:323–332.

2. Tseng KC, Lin CW, Tzeng JE, Feng WF, Hsieh YH, Chou AL, et al. Gardner's syndrome--emphasis on desmoid tumors. Tzu Chi Med J. 2006. 18:57–60.

3. Gardner EJ. A genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum. Am J Hum Genet. 1951. 3:167–176.

4. Béroud C, Soussi T. APC gene: database of germline and somatic mutations in human tumors and cell lines. Nucleic Acids Res. 1996. 24:121–124.

5. Lin RC, Chiu HH, Jao YTFN, Liu YW, Huang TC, Mo LR, et al. Carcinoma of ampulla of vater and recurrent desmoid tumors in Gardner's syndrome. J Intern Med Taiwan. 2008. 19:158–163.

6. Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. 2001. 27:701–706.

7. Rampone B, Pedrazzani C, Marrelli D, Pinto E, Roviello F. Updates on abdominal desmoid tumors. World J Gastroenterol. 2007. 13:5985–5988.

8. Zissiadis A, Harlaftis N, Aletras H. Desmoid tumor in Gardner's syndrome. Am Surg. 1990. 56:305–307.

9. Hatzimarkou A, Filippou D, Papadopoulos V, Filippou G, Rizos S, Skandalakis P. Desmoid tumor in Gardner's syndrome presented as acute abdomen. World J Surg Oncol. 2006. 4:18.

10. Belliveau P, Graham AM. Mesenteric desmoid tumor in Gardner's syndrome treated by sulindac. Dis Colon Rectum. 1984. 27:53–54.

11. Lee BD, Lee W, Oh SH, Min SK, Kim EC. A case report of Gardner syndrome with hereditary widespread osteomatous jaw lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009. 107:e68–e72.