Tumors of the pancreas can either be benign (non-cancerous) or malignant (cancerous). There are certain tumors that are classified as atypical (uncertain or unspecified behavior) meaning that some behave in a benign manner, or some behave in a malignant manner. A majority of the pancreatic tumors diagnosed via lab studies (biopsy) are benign.

The World Health Organization (WHO) is an authoritative body that compiles, prepares, and publishes histological and molecular classification of tumors, via their Blue Books, based on research inputs from experts worldwide. These reference books chiefly aid in global cancer research, provide gold standards for tumor diagnosis, and help in treating cancer patients.

According to the WHO Classification of Digestive System Tumors (2019), 5th Ed., Vol. 1, benign and malignant tumors of the pancreas are classified in the following manner:

Tumors of the pancreas:

Epithelial tumors

• Benign epithelial tumors and precursors
  • Acinar cystic transformation of the pancreas: It is an uncommon benign condition of the pancreas. Some research indicates that the condition may be caused by genetic factors
  • Serous neoplasms of the pancreas: These are benign or malignant neoplasms of the pancreas, which are mostly observed in women. There are two main variants - serous cystadenoma and serous cystadenocarcinoma
  • Pancreatic intraepithelial neoplasia: It is an abnormality in the pancreatic epithelium, which results in dysplasia of the epithelial lining of the duct. The condition is more prevalent in older individuals
  • Pancreatic intraductal papillary mucinous neoplasm: It is a pancreatic tumor that typically arises from the pancreatic duct. The condition is more prevalent in older adults (over 60 years of age)
  • Pancreatic intraductal oncocytic papillary neoplasm: It is a tumor arising in the exocrine pancreas. It typically occurs in elderly adults. Most of the tumors behave in a benign manner, while some are known to be invasive
  • Pancreatic intraductal tubulopapillary neoplasm: It is a very rare benign tumor that forms in the exocrine portion of the pancreas. It typically occurs in older adults
  • Pancreatic mucinous cystic neoplasm: It is an exocrine, benign or precancerous lesion, typically found in the body and tail of the pancreas. It predominantly affects women aged 40 years or older
• Malignant epithelial tumors
  • Pancreatic ductal adenocarcinoma: It is the most common type of pancreatic cancer. The malignancy typically affects middle-aged and older individuals
  • Pancreatic acinar cell carcinoma: It is a rare, malignancy of the acinar cells of the pancreas. It typically occurs in elderly men
  • Pancreatoblastoma: It is a rare, malignant tumor the pancreas. Though it is a rare tumor, it is the most common pancreatic tumor of infancy and early childhood
  • Solid pseudopapillary neoplasm of the pancreas: It is a type of exocrine tumor of the pancreas, with a low potential for malignancy. The tumor is almost exclusively observed in adolescent girls and women
• Pancreatic neuroendocrine neoplasms
  • Pancreatic neuroendocrine neoplasms
  • Non-functioning pancreatic neuroendocrine tumors: These may be described as malignant epithelial cancers involving the pancreas. Both children and adults are observed to have these tumors
• Functioning pancreatic neuroendocrine tumors
  • Insulinoma: It is a rare tumor of the beta cells of the pancreas, which secretes insulin. They are considered to be functionally-active tumors because they secrete insulin, causing ‘clinical hypoglycemia’
  • Gastrinoma: It is a malignant tumor of G-cells that produce the hormone known as gastrin. These tumors are considered to be functionally-active tumors, owing to the hormone they secrete. They are more common among adults, particularly men
  • VIPoma: It is an extremely rare endocrine tumor of pancreas that secretes vasoactive intestinal peptide. The tumor generally occurs in young and middle-aged adults
  • Glucagonoma: It is a malignant tumor caused by an increase in glucagon hormone produced by alpha cells of the pancreas. This leads to a condition known as glucagonoma syndrome. These tumors usually occur in middle-aged women
  • Somatostatinoma: It is a very rare, malignant tumor arising from the delta cells of the pancreas (from the endocrine portion of the pancreas). It generally occurs in middle-aged and slightly older individuals
  • ACTH-producing neuroendocrine tumor: It is a subtype of functioning pancreatic neuroendocrine tumor (PanNET). The tumor observed in young and middle-aged adults, mostly women
  • Serotonin-producing neuroendocrine tumor
• Pancreatic neuroendocrine carcinoma: It is a malignant and aggressive tumor that originates from the neuroendocrine cells. The cancer is usually seen in adults
• Pancreatic MiNENs: These are malignant epithelial tumors involving the pancreas, an important organ of the digestive system located in the abdomen. These are observed in both men and women

Additional pancreatic tumor articles (on DoveMed) that are not part of WHO Classification of Digestive System Tumors:

Acinar Cell Cystadenocarcinoma of Pancreas: It is a rare cancer of the acinar cells of the pancreas that forms malignant cysts. It typically occurs in middle-aged and elderly adults.

Acinar Cell Cystadenoma of Pancreas: It is a rare and benign cystic lesion of the pancreas, typically lined by cells exhibiting acinar cell like properties. The lesion can occur in individuals of all ages.

Adenosquamous Carcinoma of Pancreas: It is a rare, malignant tumor of the pancreas having malignant squamous cells and glandular cells. It typically affects older men.

Benign Mucinous Cystadenoma of Pancreas: It is a slow-growing and rare pancreatic tumor. In this tumor, the lining of the cyst consists of mucin-producing cells. It generally occurs in women

Clear Cell Pancreatic Endocrine Tumor: It is a rare, malignant endocrinal tumor of the pancreas. It typically occurs in adults, commonly in association with genetic disorders von Hippel-Lindau (vHL) syndrome or multiple endocrine neoplasia type 1 (MEN 1).

Colloid Carcinoma of Pancreas: It is a malignant cancer of the pancreas that secretes mucin (a type of protein). It generally occurs in older individuals.

Dermoid Cyst of Pancreas: It is a very rare and benign tumor/cyst of the pancreas. It typically occurs in young adults, though some cases have been observed in children.

Desmoplastic Small Round Cell Tumor (DSRCT) of Pancreas: It is a very rare, soft tissue tumor of the pancreas. It is a type of high-grade malignant cancer that spreads and infiltrates into the surrounding tissues and organs. It is more common in children, adolescents, and young adults.

Diffuse Large B-Cell Lymphoma (DLBCL) of Pancreas: It is a fast-growing, malignant and non-Hodgkin lymphoma of the B-cells. The malignancy is generally reported in older men and women.

EC Serotonin-Producing NET of Pancreas: It is a very rare and malignant tumor that generally affects middle-aged adults. It is associated with carcinoid syndrome from serotonin production.

Enterogenous Cyst of Pancreas: It is very rare benign tumor of the pancreas that forms as a cyst. It typically occurs in young adults.

Ewing Sarcoma (ES) of Pancreas: It is a malignant tumor of the pancreas, which arises from primitive cells. Although the sarcoma can arise at any age, it is more common in teens and young adults.

Inflammatory Myofibroblastic Tumor of Pancreas: It is a very rare pancreatic tumor consisting of spindle cells, mixed lymphocytes, and plasma cells. It may arise in individuals of any age.

Inherited Pancreatic Cancer: It is a general term to describe the occurrence of an abnormally high rate of pancreatic cancers in certain families. The manifestation of signs and symptoms may occur in adulthood.

Intraductal Papillary Mucinous Neoplasm (IPMN) of Pancreas: It is an exocrine, cystic tumor that grows within the pancreatic duct. The tumor affects older men and women.

Intraductal Tubular Adenoma of Pancreas: It is an uncommon benign tumor of the pancreas that arises within the main pancreatic duct. It typically occurs in elderly adults.

Large Cell Neuroendocrine Carcinoma of Pancreas: It is a rare form of malignancy that is observed to develop in adults. This type of malignant tumor can occur anywhere in the pancreas and may be associated with excess synthesis of hormones.

Lipoma of Pancreas: It is a benign tumor of the pancreas consisting of fat tissues. It typically occurs in middle-aged and older adults.

Lymphangioma of Pancreas: It is a rare, benign tumor of the pancreas that develops spontaneously due to certain abnormalities in the lymphatic system. It typically occurs in newborns and very young children.

Lymphoepithelial Cyst (LEC) of Pancreas: It is a rare and benign cyst of the pancreas. It typically occurs in middle-aged and older men.

Macrocystic Serous Cystadenoma of Pancreas: It is a common benign tumor of the pancreas that forms as a cyst. It typically occurs in elderly men.

Medullary Carcinoma of Pancreas: It is a very rare malignant pancreatic tumor. It generally occurs in middle-aged and elderly adults.

Mixed Acinar-Ductal Carcinoma of Pancreas: It is a rare and aggressive type of pancreatic cancer characterized by a mixed population of cells from both acinar and ductal origin within the tumor. These are generally seen in middle-aged and older adults.

Mixed Acinar-Neuroendocrine Carcinoma (MANEC) of Pancreas: It is an extremely rare type of pancreatic cancer characterized by a mixed population of cells having both exocrine and endocrine origin within the tumor. The condition is known to affect older individuals.

Mixed Ductal-Neuroendocrine Carcinoma of Pancreas: It is a rare and aggressive type of pancreatic cancer characterized by a mixed population of cells from both the exocrine and endocrine region. These carcinomas are typically observed in adults.

Mixed Osteoclastic and Pleomorphic Giant Cell Tumor (GCT) of Pancreas: It is a rare, malignant pancreatic tumor of the exocrine pancreas. It occurs in middle-aged and older adults.

Mucinous Cystadenocarcinoma of Pancreas: It is a malignant pancreatic tumor. In this tumor, the lining of the malignant cyst consists of mucin-producing cells. It generally occurs in women.

Neuroendocrine Tumor of Pancreas: It is a tumor that arises from the neuroendocrine cells of the pancreas. It is a rare tumor type that is seen in older adults.

Osteoclastic Giant Cell Tumor (GCT) of Pancreas: It is a rare, malignant pancreatic tumor of the exocrine pancreas that typically occurs in adults.

Pancreatic Cancer: It is a general term for a cancer that originates in pancreatic tissues. It is a common type of cancer that affects men and women worldwide.

Pancreatic Hepatoid Carcinoma: It is a rare, malignant tumor the pancreas that affects adults. On histological examination of the tumor, it appears like liver tissues, and hence termed ‘hepatoid’.

Pancreatic Neuroendocrine Microadenoma: It is a biologically benign tumor in the hormone-producing cells of the pancreas. It is predominantly reported in older adults.

Pancreatic Pseudocyst: It is a fluid-filled sac that develops on the pancreas, usually due to pancreatitis. They contain pools of pancreatic fluid, debris, and destroyed tissue. It is a predominantly benign condition.

Perivascular Epithelioid Cell Neoplasm (PEComa) of Pancreas: It is a soft tissue tumor in the pancreas that shows perivascular epithelioid-cell (PEC) differentiation. Such tumors can be benign or malignant. This tumor type is known to develop in adults.

Pleomorphic Giant Cell Tumor (GCT) of Pancreas: It is a rare, malignant tumor of the exocrine pancreas. It generally occurs in elderly men and women.

Primitive Neuroectodermal Tumor (PNET) of Pancreas: It is a rare, malignant, and life-threatening pancreatic tumor. It generally occurs in young individuals.

Pseudopapillary Tumor of Pancreas: It is a rare low-grade malignancy that forms in the exocrine pancreas. It is mostly seen in middle-aged women.

Serous Cystadenocarcinoma of Pancreas: It is a very rare, malignant cancer of the pancreas that forms as a cyst. It typically occurs in elderly adults.

Serous Cystadenoma of Pancreas: It is an uncommon benign tumor of the pancreas that forms as a cyst. It typically occurs in women.

Signet Ring Carcinoma of Pancreas: It is a very rare and aggressive form of pancreatic cancer. It generally affects older men and women.

Small Cell Neuroendocrine Carcinoma of Pancreas: It is a rare malignant tumor of the pancreas that may or may not be associated with excess synthesis of hormones. It is observed to develop in adults.

Solid Serous Cystadenoma of Pancreas: It is a very rare, benign tumor of the pancreas that forms as a combination of cyst and solid portions. It typically affects adults.

Solitary Fibrous Tumor (SFT) of Pancreas: It is a mostly benign overgrowth arising from mesenchymal tissue. It is usually diagnosed in the fifth decade.

Undifferentiated Carcinoma of Pancreas: It is a type of malignant exocrine tumor of the pancreas, in which the biological origin of most cells in the tumor is unclear. It typically affecting older adults.

Von Hippel-Lindau Associated Serous Cystic Neoplasm of Pancreas: It is a tumor of the pancreas that occurs in the background of von Hippel-Lindau syndrome (an inherited genetic disorder). It typically affects young adults.