In Switzerland, around 1,400 people are diagnosed with pancreatic cancer every year, mainly ductal adenocarcinoma, which leads to around 1,150 deaths. Typical symptoms include unwanted weight loss, abdominal pain and jaundice. Diagnosis is usually made by CT or MRI/MRCP followed by endosonography. The standard treatment consists of surgical removal of the tumor followed by adjuvant chemotherapy and radiotherapy. The prognosis is often unfavorable, as the disease is often already well advanced at the time of initial diagnosis.
Most pancreatic carcinomas are exocrine tumors that arise from ductal and acinar cells. Adenocarcinomas of the exocrine pancreas develop nine times more frequently from ductal than from acinar cells, with around 80% occurring in the head of the pancreas. On average, adenocarcinomas manifest themselves around the age of 55, with men being affected 1.5 to 2 times more frequently than women. Endocrine pancreatic tumors are another tumor entity with a better prognosis.
Prominent risk factors for pancreatic cancer include
- Smoking
- previous episodes of chronic pancreatitis
- Obesity
- male gender
Heredity also plays a role, while alcohol and caffeine consumption do not appear to be risk factors.
What can be symptoms and signs of pancreatic cancer?
Pancreatic cancer, primarily ductal adenocarcinoma, is responsible for approximately 57,600 cases and 47,050 cancer deaths annually in the United States. Symptoms include unintentional weight loss, abdominal pain and jaundice. Diagnosis is made by CT or MRI/MRCP and then by endosonography. Treatment usually consists of surgical resection and adjuvant chemotherapy and radiotherapy. The prognosis is poor, as the disease is often already advanced when it is first diagnosed.
Most pancreatic carcinomas are exocrine tumors that develop from ductal and acinar cells. Adenocarcinomas of the exocrine pancreas originate 9 times more frequently from ductal than acinar cells; 80% occur in the head of the pancreas. Adenocarcinomas occur on average around the age of 55 and are 1.5 to 2 times more common in men than in women.
Prominent risk factors for pancreatic cancer include smoking, a history of chronic pancreatitis, obesity, being male and being black. Heredity plays a certain role. Alcohol and caffeine consumption do not appear to be risk factors.
What are the symptoms and signs of pancreatic cancer?
The symptoms of pancreatic cancer, such as pain and weight loss, are often non-specific, leading to a delayed diagnosis, especially if the disease has already spread. At initial diagnosis, about 90% of patients already have locally advanced tumor disease that affects retroperitoneal structures, spreads to regional lymph nodes or metastasizes to the liver or lungs.
Typically, most patients suffer from severe upper abdominal pain, which often radiates to the back. Weight loss is also common. Adenocarcinomas in the head of the pancreas cause obstructive jaundice in 80-90% of patients, which is often accompanied by itching. Carcinoma in the body or tail of the pancreas can cause blockage of the splenic veins, leading to enlargement of the spleen, gastric and oesophageal varices and gastrointestinal bleeding.
Between 25-50% of patients develop diabetes mellitus due to the carcinoma, leading to symptoms such as increased urination and thirst. In some patients, pancreatic cancer can also impair the production of digestive enzymes (exocrine pancreatic insufficiency), which leads to problems with the absorption of nutrients. This can cause bloating, gas formation and watery, greasy and/or foul-smelling diarrhea, which ultimately leads to weight loss and vitamin deficiencies.
How is pancreatic cancer detected?
- CT or magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP)
- Endosonography
CA 19-9 antigen is used for progression assessment but not for early detection, and the preferred investigations include abdominal spiral CT with pancreatic technique or MRI/MRCP followed by endoscopic ultrasonography with fine needle aspiration (EUS/FNA or FNB) for tissue diagnosis and assessment of surgical resectability.
The choice between CT and MRI/MRCP often depends on local availability and expertise. Even if these imaging modalities show inoperable or metastatic disease, EUS/FNA or percutaneous needle aspiration is performed to obtain a tissue diagnosis. If CT shows potentially resectable or no tumor, MRI/MRCP or endosonography may be used for staging or to identify smaller lesions not visible on CT. In patients with obstructive jaundice, endoscopic retrograde cholangiopancreatography (ERCP) may be the first diagnostic procedure. Routine laboratory tests should be performed. An increase in alkaline phosphatase and bilirubin indicates bile duct obstruction or liver metastases. The pancreas-associated antigen CA 19-9 can be used to monitor patients diagnosed with pancreatic cancer and to screen high-risk patients. However, this test is not sensitive or specific enough for screening in the general population. Elevated levels should decrease with successful treatment; a later increase indicates progression. Amylase and lipase levels are usually not elevated.
What is the prognosis of pancreatic cancer?
The prognosis for pancreatic cancer varies depending on the stage, but overall survival is poor (5-year survival: < 2%) as many patients are already at an advanced stage of the disease at the time of diagnosis.
How is pancreatic cancer treated?
- Whipple procedure (pancreaticoduodenectomy)
- adjuvant chemotherapy and radiotherapy
Approximately 80-90% of cases are considered non-resectable at initial diagnosis, often due to metastases or invasion of large vessels. The choice of treatment depends on various factors, but the Whipple procedure is often the preferred method for resection of the tumor, especially in the pancreatic head. Adjuvant therapies, such as gemcitabine-based combinations, are recommended to prolong survival.
If a non-resectable tumor is detected during surgery and obstruction is present, bypass surgery can be performed to relieve symptoms. For inoperable tumors and jaundice, endoscopic or endosonography-guided stent placement can help improve quality of life. Treatment of pain and other symptoms is crucial and includes the use of analgesics, maintenance of biliary patency and pancreatic enzyme supplementation.
Palliative therapy aims to improve quality of life and relieve symptoms. Pain can be controlled with oral opioids, and coeliac plexus blocks can be considered if necessary. Itching due to obstructive jaundice can be treated with cholestyramine.
What are special forms of pancreatic cancer?
- Cystadenocarcinoma: Cystadenocarcinoma is a rare adenomatous pancreatic carcinoma that arises as a malignant transformation of a mucinous cystadenoma and usually presents with upper abdominal pain and a palpable abdominal mass.
The diagnosis of cystadenocarcinoma is made by CT or MRI of the abdomen, which typically show a cystic mass containing necrotic tissue; the mass may be misinterpreted as a necrotic adenocarcinoma or pancreatic pseudocyst.
In contrast to ductal adenocarcinoma, cystadenocarcinoma has a relatively good prognosis. Only 20% of patients have metastases at the time of surgery; complete tumor excision by distal or total pancreatectomy or Whipple procedure leads to a 5-year survival of 65%. - Intraductal papillary mucinous tumor: Intraductal papillary mucinous tumor is a tumor that leads to hypersecretion of mucus and obstruction of the duct. The histology can be benign, borderline or malignant. Most tumors occur in women (80%) and in the tail of the pancreas (66%). The symptoms of intraductal papillary mucinous tumor consist of pain and repeated attacks of pancreatitis. The diagnosis of an intraductal papillary mucinous tumor is made by CT or MRI. Surgical resection is the treatment of choice for patients with intraductal papillary mucinous tumors with high-grade dysplasia that have progressed to invasive carcinoma or have features that indicate a high risk of cancer. With surgical resection, 5-year survival is > 95% for benign or borderline cases, but 50-75% for malignant tumors.