Islet cell carcinoma is a rare disease affecting pancreatic tissue cells, which can be functional or non-functional. Tumors are categorized based on the type of pancreatic endocrine cells affected and symptoms vary. Diagnosis is made through laboratory and imaging tests, and treatment depends on the number and location of tumors.
Islet cell carcinoma, also known as islet cell cancer, is a relatively rare disease that produces abnormal pancreatic tissue cells. Islet cell cancer is also sometimes called pancreatic carcinoid, pancreatic endocrine tumor (PET), or islet cell carcinoma. These tumors fall into several distinct categories based on the types of pancreatic endocrine cells they affect. Whether there are symptoms and whether the nature of those symptoms varies considerably depends on the status of the tumor as functional or non-functional. Diagnosis is usually made after laboratory and imaging tests.
Pancreatic endocrine cells produce hormones that control the functions of organs and cells throughout the body. These cells cluster in the pancreas to form islets, also known as the islets of Langerhans. Islet cell tumors are referred to as functional tumors if their presence produces symptoms or nonfunctional in cases where the tumor causes no symptoms. Most functional tumors are benign, while most nonfunctional tumors are cancerous.
The specific types of islet cell carcinoma tumors are based on the hormones produced by the affected cells. A gastrinoma is a tumor that forms in the cells of the pancreas that produce gastrin, a hormone that triggers the release of stomach acid and thus aids in digestion. Most commonly found in the head of the pancreas, gastrinomas are usually malignant and often cause diarrhea. A glucagonoma tumor forms in cells that produce glucagen, a hormone that raises blood glucose levels, and these tumors often cause hyperglycemia. Most glucagonoma tumors are cancerous.
Insulinoma is a tumor that grows in cell tissue that produces insulin which produces hormones that control the flow of glucose into the body’s cells, providing energy. Insuloma tumors can develop anywhere in the pancreas and are commonly benign. VIPoma tumors affect the production of hormones that control the balance of water, sugar and salt in the body, while somatostatinomas affect cells that produce somatostatin, a hormone that regulates cell proliferation and neurotransmission.
Nonfunctional islet cell carcinoma can take many years to develop and often causes no symptoms until it reaches a size that causes mechanical difficulties in the body. General symptoms may include pain in the back or abdomen, indigestion, diarrhea, or a lump in the abdomen. Functional tumors produce symptoms based on the affected hormone production. These include recurring ulcers and digestive tract disease, as well as symptoms of low or high blood sugar. Unlike other forms of cancer, there are no standardized stages assigned to islet cell carcinoma; treatment is based on the number and location of tumors and may consist of radiation or chemotherapy.
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