A Case Report of Gardner's Syndrome and a Review of Literature
Volume
5
(2010) Issue
3 (Summer )
Case Report
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KeyWords:
Gardner’s Syndrome, Familial Polyposis Coli, Abdominal Fibromatosis, Iran
Authors:
Mitra Heidarpour, Seyed Abass Tabatabai , Majid Heidarpour , Farzaneh Sajjadi
Abstract:
Gardner’s syndrome is an autosomal dominant inherited disorder. Familial polyposis of the
colon, osteomas, hypertrophy of the retinal-pigmented layer and a multitude of soft tissue tumors
are characteristic features. The syndrome may be presented with colonic or extracolonic symptoms.
A 75-year-old male patient presented to Al-zahra Clinic with diffuse abdominal pain. An abdominal
surgery was performed on him due to invasive abdominal mass. The surgical specimen was
examined by H&E and immunohistochemical staining. The final diagnosis was fibromatosis. There
was a history of gardner’s syndrome in his family. Colonoscopy was done to confirm the diagnosis
of gardner’s syndrome. The diagnosis of gardner’s syndrome was made according to following
findings: abdominal fibromatosis, multiple jaw osteomas and polyposis coli. The patient with
gardner’s syndrome can present with abdominal fibromatosis even in an old age. In IHC staining
CD117 was seen with coarse granular cytoplasmic pattern in fibromatosis, and so, this pattern of
CD117 staining can be a clue to the diagnosis of fibromatosis.
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