No abstract available.
Gardner Syndrome*

No abstract available.
Gardner Syndrome* ; Rectal Neoplasms*

Pilomatricoma is a benign tumor derived from the hair matrix. Pilomatricoma-like changes have been described in epidermal cysts in Gardner's syndrome. Apart from this association, a cyst can arise from various portions of a hair follicle, and combine to form a hybrid cyst. Follicular hybrid cyst is an interesting pathologic phenomenon, and needs to be explained for their pathogenesis. We present a case in which a pilomatricoma was situated in the immediate vicinity of an epidermal cyst. Histopathologic findings showed that one part of the cyst had a feature of an epidermal cyst, and the other part had a feature of a pilomatricoma.
Chimera ; Epidermal Cyst ; Gardner Syndrome ; Hair ; Hair Follicle ; Pilomatrixoma

Hyperplastic polyps occur either sporadically or as a symptom of polyposis syndrome. When individuals exceed 50 polyps, they are diagnosed with hyperplasic polyposis. Moreover, since such cases are even more sporadic than hyperplastic polyps, the course toward this occurrence has not been properly evaluated. A change to malignancy in hyperplastic polyps is rare; however, when multiple lesions are present, the tendency increases. Colorectal polyposis syndromes with gastric polyps include familial adenomatous polyposis, Gardners syndrome, Peutz-Jeghers syndrome, juvenile polyposis and others with a non-genetic origin. Three cases of multiple colorectal hyperplastic polyposis with gastric hyperplastic polyposis have been reported worldwide; however, a case associated with multiple colonic adenomas has not yet been reported. This study reviews the existing literature and reports our recent experience of a case, in which a 53 year-old man with colorectal and gastric hyperplastic polyposis with associated multiple colonic adenomas.
Adenoma* ; Adenomatous Polyposis Coli ; Colon* ; Gardner Syndrome ; Humans ; Hyperplasia ; Middle Aged ; Peutz-Jeghers Syndrome ; Polyps

Hyperplastic polyps are a benign epithelial proliferation and a common type of colorectal and gastric polyps. Malignant change of hyperplatic polyps is rare. Patients with multiple lesions however, tend to have a high frequency of malignancy. Colorectal polyposis syndromes with gastric polyps include familial adenomatous polyposis, Gardners syndrome, Peutz-Jeghers syndrome, juvenile polyposis and others. But the case of colorectal hyperplastic polyposis with multiple gastric hyperplastic polyps has not been reported until now. A case was recently experienced involving a 32 year old women with both colorectal and gastric hyperplastic polyposis. Multiple hyperplastic polyps were found in the lower part of the body and the antrum of the stomach, rectosigmoid, transverse and ascending colon.
Adenomatous Polyposis Coli ; Adult ; Colon, Ascending ; Female ; Gardner Syndrome ; Humans ; Peutz-Jeghers Syndrome ; Polyps* ; Stomach*

Gastric polyps are uncommon. The incidence of gastric polyps has been reported between 0.4% and 2.0%. Two histologically distinct forms of gastric polyps are hyperplastic polyps and adenomatous polyps. Hyperplastic polyps are multiple in up to a half of cases. When the polyps number is more than 50, the term "Hyperplastic polyposis" is applied and such cases are even more rare. Polypoid lesions of the stomach have heen reported in many of the polyposis syndromes, such as Familial polyposis coli, Gardners syndrome, Peutz-Jeghers syndrame, Juvenile polyposis and Cowdens disease. Only 1 case of gastric hyperplastic polyposis associated with colonic hyperplastic polyposis has been reported. The patient under study is not included in any polyposis syndrome and has no familial tendency, We report a case of gastric hyperplastic polyposis with colonic hyperplastic polyposis with literatures reviews.
Adenomatous Polyposis Coli ; Adenomatous Polyps ; Colon* ; Gardner Syndrome ; Hamartoma Syndrome, Multiple ; Humans ; Incidence ; Polyps ; Stomach

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

Mesenteric fibromatosis is a rare nonmetastasizing fi brous tumor, characterized by fibroblastic proliferation without evidence of inflammatory response or definite neoplasia. It appears at any age, but mainly in the second to fifth decades as incidence of two to four desmoid tu mor cases per 1 million people per annum. It is pseudoen capsulated and locally invasive and has a tendency to recur, but metastases are rare. It is more frequent with Gardner's syndrome(adenomatosis coli) in which 50% of them will appear as mesenteric fibromatosis. Trauma, especially that of previous surgery, has been associated with the subsequent development of desmoid tumor. The treatment of choice is a wide margin resection of the tumor. we experienced a case of mesenteric fibromatosis without Gardner's syndrome 2 years after appendectomy in a 31-year old man and recurred at 21 months after resection and report with a review of literatures. 6
Adult ; Appendectomy* ; Fibroblasts ; Fibroma* ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Incidence ; Neoplasm Metastasis

Congenital hypertrophy of the retinal pigment epithelium (CHRPE)is a well circumscribed, flat, pigmented fundus lesion that have a variety of shapes. It is the most common extracolonic manifestation of Gardner's syndrome. The number and shape of CHRPE should arouse suspicion of increased risk of polyposis.We experienced one case of Gardner's syndrome in a family history of colonic carcinoma that showed atypical congenital hypertrophy of the retinal pigment epithelium, osteoma, epidermal cyst and polyposis combined with colonic carcinoma.A total colectomy and ileostomy were performed.So we reported it with the review of literature of clinical value of CHRPE as a predictive marker of Gardner's syndrome.
Colectomy ; Colon ; Epidermal Cyst ; Gardner Syndrome* ; Humans ; Hypertrophy* ; Ileostomy ; Osteoma ; Retinal Pigment Epithelium* ; Retinaldehyde*

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) has been described in association with systemic disorders such as Gardner's syndrome (intestinal polyposis, osteomas, and benign soft-tissue tumors), Turgot's syndrome (glioma-polyposis syndrome) and familial adenomatous polyposis (FAP) of the colon. FamiIial adenomatous polyposis is an autosomal dominant disorder characterized by hundreds of polyps throughout the entire colon, and in all patients carcinoma of the color, develops at the age of 40 to 50 years. We describe a family with familial adenomatous polylpcsis coli and congenital hypertrophy of the retinal pigment epithelium. All of them except mother showed flat, weII-demarcated, round to oval pigmented patches of CHRPE. We emphasize the importance of CHRPE as a clinical marker in identifying patients With FAP since they are at risk for cancer.
Adenomatous Polyposis Coli* ; Biomarkers ; Colon ; Gardner Syndrome ; Humans ; Hypertrophy ; Mothers ; Osteoma ; Polyps ; Retinal Pigment Epithelium* ; Retinaldehyde*