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Gastric cancer is the third most common cancer worldwide. The majority of gastric cancers are sporadic but familial clustering is seen in more than 10% of cases. This manuscript is divided into two parts. The first part is dedicated to the non-syndromic hereditary gastric cancer, particularly the hereditary diffuse gastric cancer (HDGC) and other gastric polyposes including the recently described GAPPS (Gastric adenocarcinoma and proximal polyposis of the stomach). The second part concerns the syndromic gastric cancer, namely the HNPCC syndrome (Hereditary Non Polyposis Colorectal Cancer) occurring as part of a genetic predisposition syndrome to cancer. Recent advances in oncogenetics and next generation sequencing technology have enabled the identification of new entities. This enhancement in knowledge regarding inherited syndromes predisposing to gastric cancer has consequently improved the management of patients and their families. In this context, pathologists play a major role in identifying particular morphologic entities prompting genetic investigation. The aim of this manuscript is to provide an update on the current knowledge about hereditary gastric cancer.

We report one case of adenomatous polyposis of the gallbladder in a 57 year-old woman with Gardner's syndrome presenting with cholangitis. On gross examination the gallbladder contained two calculi and numerous flat or polypoid adenomas less than 1 cm in size. On microscopic examination, the adenomas showed low and high grade intraepithelial neoplasia. Only 10 cases of gallbladder adenomas have been reported in the literature in patients presenting with familial adenomatous polyposis (FAP). Cholecystectomy is usually performed for cholecystitis or cholangitis. These adenomatous gallbladder lesions are discovered late, often when the patient is older than 40. Pathogenesis of gallbladder adenomas is still unclear. It is difficult to assess the risk of malignancy: only 6 cases of gallbladder adenocarcinomas have been reported in patients with FAP.

Adenomas are rarely diagnosed in the appendix and may be isolated or may coexist with other neoplasms in the gastrointestinal tract. This emphasizes the need for postoperative colonoscopy when a polyp of the appendix is found. Moreover, the polyps are considered to be premalignant lesions.Report two new cases of adenomas of the appendix.We report the cases of 23-year-old and 22-year-old men, for whom appendicectomy performed for acute appendicitis. In both cases, histologic studies revealed adenomas in moderate dysplasia ; colonoscopy did not reveal any polyps.

In a retrospective study of the specimens of gastric polypectomy, carried out between January 1992 and December 2002, we analysed the demographic and histological aspects of 65 polyps. This study revealed hyperplastic polyps in 66%, adenomatous lesions in 9.2%, Peutz-Jeghers polyps in 7.7%, inflammatory fibroid polyps in 6.15%, two cases of focal foveolar hyperplasia (3%), two cases of Brunner's gland heterotopia (3%), 1 pancreatic heterotopia (1.5%), 1 fundic gland polyp (1.5%) and 1 carcinoid tumor (1.5%). All adenomas and two Peutz-Jeghers polyps include intraepithelial neoplasia. Moreover, we identified a case of Brunner's gland heterotopia, which contain a focus of plane tubular adenoma with high-grade intraepithelial neoplasia. Other lesions were found within the polyps or into the surrounding gastric mucosa, such as intestinal metaplasia and Helicobacter Pylori gastritis. This work allowed us to recommend complete removal of gastric polyps and the realization of biopsies of the nonpolypoid gastric mucosa in the search of intraepithelial neoplasia or other lesions with malignant potential.

Turcot's syndrome is characterized clinically by the concurrence of a primary brain tumor and a familial adenomatous polyposis or a hereditary nonpolyposis colorectal cancer.We report a case of a 45-year-old woman who underwent in 1995 neuro-oncological treatment for an anaplastic astrocytoma (grade III according to the World Health Organization classification). Treatment included complete surgery, radiotherapy, a first-line nitrosourea-based chemotherapy regimen and a second-line platinium salt-based regimen. It was then noted that the patient's brother had colorectal cancer. A genetic study detected a germ-line mutation on the hMSH2 gene specific of HNPCC syndrome (Human Non Polyposis Colorectal Cancer). Colonoscopy was normal. Eight years after the diagnosis, the patient developed a gliomatosis cerebri and died.Relevant personal and familial history can provide the clue to the diagnosis of Turcot's syndrome. Molecular diagnosis may contribute to appropriate care of affected patients.

A case of gastric hyperplastic polyposis is reported in a 48-year old woman, with iron deficiency anemia. An hyperplastic gastric polyposis was discovered. This patient had been operated 17 years previously for a large adenomatous polyp of the caecum. Her son had also several adenomatous polyps of the right colon. A gastrectomy was performed. Hyperplastic gastric polyposis is very rare, and is quite always associated with colorectal adenomas. The relationship between gastric hyperplastic polyposis and intestinal polyposis is not quite clear.

Hyperplastic polyps are the most common type of colorectal polyps. They are usually multiple and localized in the rectosigmoid area. They are easily recognized by their small size (10 mm), sessile form, smooth-surface and translucid appearance. Typically, these polyps have no malignant potential. We report the case of a 68-year-old man who had multiple rectosigmoid polyps, some of them having macroscopic features of adenomatous polyps. Because of the occurrence of bleeding after endoscopic polypectomy of two polyps corresponding to adenomas at examination, because of the multiplicity of these polyps and their localization in diverticulosis, a left colectomy followed by coloanal anastomosis was performed. Histologic examination of the surgical specimen revealed the hyperplastic nature of all polyps with, in two of them, a focus of adenomatous tissue.

Eight cases of pneumatosis cystoides intestinalis (PCI) affecting the descending colon at first misdiagnosed in seven of them, are reported. The mechanism of cyst formation remains unclear but certain specific endoscopic and X-ray findings should help in differentiating PCI from other intramural tumors, i.e. polyposis. Diagnostic investigations and management approaches are described. No treatment is necessary in most instances because of the benign nature of PCI.