The Pulse - NOVEMBER  2005

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What is Pancreatic Cancer?

Cancer of the pancreas has a increased dramatically over the last fifty years and now ranks as the fourth leading cause of cancer death in the United States. Despite the high mortality rate associated with pancreatic cancer, the causes and progression of the disease is poorly understood. According to the National Cancer Institute, cancer of the pancreas is rarely curable and has an overall survival rate under 4%. In 2005 alone it is estimated that there were 32,180 new cases and 31,800 deaths from pancreatic cancer in the United States in 2005.

The highest cure rate occurs if the tumor is truly localized to the pancreas; however, this stage of the disease accounts for <20% of cases, early detection is critical. However since there are no tumor specific markers for pancreatic cancer, the vast majority of new cases - 90% of pancreatic cancer have progressed past this level. For patients with advanced cancers, the overall survival rate of all stages is <1% at 5 years with most patients dying within 1 year.

Symptoms

Symptoms of pancreatic cancer can be quite vague and common to many other less serious conditions. However if cancer is affecting the function of the pancreas it can potentially cause 2 problems:

• Poor absorption of food, especially fat leading to weight loss and rather fatty, pale, smelly stools and diarrhea
• Diabetes if too little insulin is produced

If the tumor is at the head of the pancreas, a majority of patients will have obstructive jaundice at the time of diagnosis, due to a tumour in the head of the pancreas obstructing the bile duct, and hence the associated symptoms (yellowing eyes and skin, dark urine, pale stools, itchy skin). The jaundice is often painless and accompanied by no other symptoms so that the patient first notices something is wrong when their skin starts itching, their urine becomes very dark and they start to turn yellow (it is noticeable first in the whites of the eyes).

For tumors that occur in the head of the pancreas and typical early symptoms can include:

• Jaundice and associated symptoms
• Feeling of sickness (nausea)
• Slight weight loss
• Unexpected development of diabetes

Tumours in the body and tail of the pancreas are more difficult to pick up early as they do not cause obstructive jaundice at an early stage. Typical symptoms can include:

• Vague abdominal pain
• Dyspepsia (indigestion)
• Stomach ulcer-like pain
• Intermittent diarrhoea
• Feeling of sickness (nausea)
• Unexplained weight loss
• Unexpected development of diabetes
• Back pain that does not go away

Forms of Pancreatic Cancer

Adenocarcinomas

This is the form of cancer that most people are talking about when they refer to "cancer of the pancreas." These tumors account for 75% of all pancreas cancers. Adenocarcinomas form glands (collections of cells surrounding an empty space.) These tumors can grow large enough to invade nerves which can cause back pain. They also frequently spread (metastasize) to the liver or lymph nodes. If this happens the tumor may be considered unresectable.

Acinar Cell Carcinomas

These rare cancerous tumors may produce excess amounts of the digestive enzymes normally produced by the pancreas. This increase in enzymes causes distinct symptoms in 20% of acninar cell carcinoma cases. Symptoms may include unusual skin rashes, joint pain and an increased level of eosinophils, a type of white blood cell.

Adenosquamous Carcinomas

This rare variant of pancreatic cancer is similar to adenocarcinoma because it also forms glands. These tumors also show "squamous differentiation." This means that the cells tend to flatten out as they grow. This variant is important to recognize because it may mimic other types of cancer that often show squamous differentiation.

Giant Cell Tumors

These tumors are extremely rare and are important to identify because they may not be as aggressive as the more common adenocarcinomas. As the name indicates, these tumors have unusually large "giant" cells. This does not mean that the tumor itself is larger than other types of tumors.

Intraductal Papillary-Mucinous Neoplasms

These rare but very distinctive tumors were first described in Japan in the 1980's. The tumors characteristically grow along the ducts of the pancreas that drain the pancreatic fluid into the small intestine.

Mucinous Cystadenocarcinomas

This is a rare, cystic, fluid containing, tumor of the pancreas. The spaces within this spongy tumor are filled with a thick fluid called mucin. Unlike the serous cystadenomas, these tumors can develop into cancer over time.

Pancreatoblastomas

These rare malignant tumors primarily occur in children. The reason for this is unknown. These tumors are referred to as the "pancreatic cancer of infancy."

Serous Cystadenomas

These rare tumors are usually benign (non-cancerous) growths. They are cystic, fluid containing, tumors with a sponge-like appearance. However, they can grow to be quite large and may need to be surgically removed if symptoms such as pain and weight loss occur. The vast majority of patients with this type of tumor are cured by its removal.

Solid and Pseudopapillary Tumors

These rare tumors occur primarily in women in their 30's. As the name implies some parts of the tumors are solid and some are papillary. These benign tumors have a very good prognosis although the tumors can spread so they should be removed completely.

Endocrine (Islet Cell) Tumors

These tumors are far less common than the non-endocrine tumors listed above. They account for about 1% of pancreatic cancers. It is very important that endocrine tumors be distinguished from non-endocrine because the treatments for the two types are very different.