Familial Adenomatous Polyposis (FAP) is a multi-tumoral syndrome that includes neoplasms in the duodenum, brain, pancreas and thyroid. The Cribriform Morular Variant (CMV) is a rare form of Papillary Thyroid Cancer seen in patients with FAP. Presented here is a 32 year old female who initially presented with an anterior neck mass followed years later by a rectal mass. She was diagnosed with FAP and colorectal adenocarcinoma and underwent total proctocolectomy with end ileostomy. She subsequently underwent a total thyroidectomy which revealed CMV Papillary Thyroid Carcinoma (CMV-PTC). Since FAP can have diverse presentations, a high index of suspicion is needed in order to make an earlier diagnosis to reduce potential morbidity and mortality. Papillary thyroid carcinoma can predate colonic polyposis. Identifying CMV-PTC early on can serve as an opportunity diagnose FAP early.

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

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*

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*

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*

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

Gardners syndrome is a single gene disorder with variable manifestations associated with the adenomatous polyposis coli (APC) gene; including gastrointestinal polyposis and osteomas associated with a variety of benign soft tissue tumors and other extraintestinal manifestations. Infrequently associated tumors include papillary carcinoma of the thyroid, biliary neoplasia and adrenal neoplasia, both benign and malignant. It is classified as a variant of classic familial adenomatous polyposis (FAP) and is an autosomal dominant disease. The incidence of incidentaloma in FAP patients is known to be higher than general population. The occurrence of an adrenal carcinoma could affect a patient's prognosis with Gardners syndrome. Increased awareness of this lesion is important in patients with Gardners syndrome who have extended lifespan after prophylactic colectomy. We report a 37 year old case of Gardners syndrome associate with adrenal adenoma.
Adenoma* ; Adenomatous Polyposis Coli ; Adrenal Gland Neoplasms ; Adult ; Carcinoma, Papillary ; Colectomy ; Gardner Syndrome* ; Humans ; Incidence ; Osteoma ; Prognosis ; Thyroid Gland

Familial adenomatous polyposis and Gardner's syndrome originate from a chromosomal abnormality, and their incidence is very rare. These diseases frequently have a familial tendency, but 10-20% do not. We experienced two cases of familial adenomatous polyposis. One had a familial tendency and was only a colonic abnormality in a 25-year-old man. The other, a 25-year-old woman had not familial tendency and lesions were consisted of colonic polyposis, a desmoid tumor of abdominal wall, and congenital hypertrophy of both retinal pigmented epithelia. We report these cases with a brief review of the literature.
Abdominal Wall ; Adenomatous Polyposis Coli* ; Adult ; Chromosome Aberrations ; Colon ; Female ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Hypertrophy ; Incidence ; Retinaldehyde

Gardner-associated fibroma (GAF) is a benign lesion of soft tissue which has recently been described and is exceedingly rare in children. GAF is associated with adenomatous polyposis coli gene mutation, familial adenomatous polyposis and desmoid. We report a case of patient with soft tissue tumor on her lower back which was turned out to be GAF. The patient was a 19-month-old female who visited out-patient clinic with palpable mass on her lower back and we performed surgical excision. The tumor was located at subcutaneous and we excised the tumor including surrounding soft tissue. She discharged without any complication on surgery day. The pathologic report showed dense collagenous tissue with spindle cell and adipose tissue, suggestive of GAF. We are planning to check gene study and to perform endoscopy and abdominal ultrasonography for at the age of 4.
Adenomatous Polyposis Coli ; Adipose Tissue ; Child ; Collagen ; Endoscopy ; Female* ; Fibroma* ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Infant* ; Outpatients ; Ultrasonography