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

• NF-PanNETs (Non-Functioning Pancreatic Neuroendocrine Tumors)
• Non-Functioning NETs of the Pancreas
• Non-Functioning Neuroendocrine Tumors of the Pancreas

What is Non-Functioning Pancreatic Neuroendocrine Tumors? (Definition/Background Information)

• Non-Functioning Pancreatic Neuroendocrine Tumors (NF-PanNETs) may be described as malignant epithelial cancers involving the pancreas, an important organ of the digestive system located in the abdomen. Over 65% of these tumors occur in the head of the pancreas
• These pancreatic neoplasms are well-differentiated neuroendocrine tumors (NETs) i.e., they show neuroendocrine differentiation, and are over 5 mm in size (by definition). If they are less than 5 mm, these are termed microadenomas which are considered to be essentially benign
• The term “non-functioning” indicate that these tumors generally do not produce excess hormones and do not present significant symptoms unless they cause obstruction of the pancreatic duct. 70-80% of the pancreatic neuroendocrine tumors (PanNETs) are the non-functioning type
• The following subtypes of NF-PanNETs are recognized:
  • Clear Cell Non-Functioning Pancreatic Neuroendocrine Tumor
  • Cystic Non-Functioning Pancreatic Neuroendocrine Tumor
  • Oncocytic Non-Functioning Pancreatic Neuroendocrine Tumor
  • Pleomorphic Non-Functioning Pancreatic Neuroendocrine Tumor
• Non-Functioning Pancreatic Neuroendocrine Tumors are highly uncommon; the prevalence is less than 2 cases per million population. Both children and adults are observed to have these tumors; age range is between 12 to 79 years, with average age of presentation at 50-55 years
• Non-Functioning Neuroendocrine Tumors of the Pancreas may occur sporadically, or they can be familial (inherited) and found along-with certain hereditary syndromes such as multiple endocrine neoplasia type 1 (MEN1). The cause of development of NF-PanNETs is not well-understood
• These may form in the pancreatic head and cause obstruction of the common bile duct and invade the small intestine (duodenum). When they form in the pancreatic tail, these tumors are known to infiltrate to the adjoining tissue structures and to the spleen. These well-circumscribed tumors may grow to about 5 cm in size
• The diagnosis of Non-Functioning Pancreatic Neuroendocrine Tumors may be challenging as they can resemble other carcinomas of the pancreatic region. A healthcare professional decides on the course of treatment, which may involve surgical excision of the tumor. Some microadenomas may progress to form malignant tumors over time

In general, individuals with malignant NF-PanNETs show a 5-year survival rate of 65–86% following surgery to remove the tumor. In those with metastatic tumors, the 5-year survival drops to 59%. The most important factor that determines the outcomes is the Ki-67 proliferation index (a reliable prognostic cell proliferation marker in cancer patients)