What are the other Names for this Condition? (Also known as/Synonyms)

• ACTH-Producing NET of the Pancreas
• ACTH-Producing Pancreatic Neuroendocrine Tumor
• Adrenocorticotropic Hormone Producing Neuroendocrine Tumor of the Pancreas

What is ACTH-Producing Neuroendocrine Tumor of the Pancreas? (Definition/Background Information)

• ACTH-Producing Neuroendocrine Tumor of the Pancreas is a subtype of functioning pancreatic neuroendocrine tumor (PanNET). It is an aggressive malignancy that may develop anywhere in the pancreas, an important organ of the digestive system located in the abdomen
• The ACTH-Producing PanNET secretes the adrenocorticotropic hormone (ACTH) resulting a condition known as Cushing syndrome, which can present weight gain, skin thinning, hypertension, increased blood glucose level, easy bruising, and weak muscles and bones
• Neuroendocrine tumors (NETs) are abnormal growth of cells that originate from the neuroendocrine cells, which are distributed throughout the body, including the pancreas. ACTH-Producing Neuroendocrine Tumor of the Pancreas is a very rare tumor observed in young and middle-aged adults (rarely in children too), with over 65% of them occurring in females
• It is reported that nearly 40% of the individuals with ACTH-Producing Pancreatic Neuroendocrine Tumor may present Zollinger-Ellison syndrome and about 5% develop insulinoma syndrome. The signs and symptoms of these may often overlap with those of Cushing syndrome
• The exact cause of tumor formation is unknown. However, the association of certain genetic tumor syndromes, such as multiple endocrine neoplasia type 1 (MEN1) or von Hippel-Lindau (vHL) syndrome, is occasionally noted. The ACTH-Producing Pancreatic Neuroendocrine Tumors can grow to large sizes (ranging from 2.5 to 15 cm in size)
• Following a diagnosis of the tumor, the treatment of ACTH-Producing Neuroendocrine Tumor of the Pancreas may involve surgery, when feasible, and other modalities such as chemotherapy and radiation therapy. However, in many cases, the outcomes are poor as over 80% of the individuals may present with metastasis at initial diagnosis