Diffuse gastric cancer

Other Names: Signet cell adenocarcinoma; Signet ring gastric carcinoma; Signet ring cell carcinoma; Signet ring cell gastric carcinoma, hereditory diffuse gastric cancer

Clinical features

Diffuse gastric cancer is a type of cancer found most often in the glandular cells lining the stomach, but can also develop in the bowel, breast, pancreas, bladder, prostate or lung.

Variants

Four major histologic patterns of gastric cancers: tubular, papillary, mucinous and poorly cohesive (including signet ring cell carcinoma), plus uncommon histologic variants. The term "signet ring cell" is often used because the cells look like signet rings when viewed under a microscope. The signet cells are a type of epithelial cell. Epithelial tissue is skin tissue, covering and lining the body both inside and out. When diffuse gastric cancer is inherited it is called "hereditary diffuse gastric cancer."

Symptoms

Signs and symptoms of gastric cancer may include indigestion, stomach discomfort, bloating, mild nausea, loss of appetite, and heartburn. In more advanced stages of gastric cancer signs and symptoms may include bloody stool, vomiting, weight loss, stomach pain, jaundice, ascites (fluid in the abdomen), and trouble swallowing.

Cause and Inheritance

Diffuse gastric cancer can be inherited or can happen sporadically in a family. Sporadic means that the cancer occurred randomly for the first time in a individual and was not inherited from a parent. Hereditary diffuse gastric cancer (HDGC) is caused by mutations in the CDH1 gene. Individuals with a CDH1 mutation typically develop cancer before age 40.

Treatment

Treatment depends on the stage at which the cancer is found and may include chemotherapy, radiation therapy, or operations to remove the stomach (gastrectomy). The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• ramucirumab (Brand name: Cyramza)Treatment of advanced gastric or gastro-esophageal junction adenocarcinoma, as a single agent or in combination with paclitaxel, after prior fluoropyrimidine- or platinium-containing chemotherapy.