- Diseases of the Pancreas
- Procedures on the pancreas:
Diseases of the Pancreas in Margate, FL
Malignant Diseases of the Pancreas
Malignant diseases of the pancreas are various types of cancers. They are initially discovered as masses in the pancreas on CT or MRI. The diagnosis of the specific type of cancer is dependent on a tissue sample. Tissue sample can be obtained before surgery by endoscopic ultrasound (EUS)-guided fine needle aspiration biopsy (FNA). Sometimes that is not possible or not practical. Then a cancer-type of operation may be necessary without an actual proof of cancer. These decisions are dependent on the individual situation of each patient.
Primary
Primary cancers of the pancreas arise from the pancreatic cells themselves. Depending on which cell type the cancer originates from, there are the following kinds:
Pancreatic Cancer
Primary pancreatic cancer originates from the pancreatic duct. It is the most common malignant tumor of the pancreas. It may form in the head of the pancreas and then it can cause jaundice. Paradoxically, development of jaundice may be the cause of earlier diagnosis of pancreatic head cancer. It may also form in the body and tail of the pancreas and then it does not cause any symptoms until late. Thus, pancreatic body cancers usually are diagnosed in more advanced stage and, because of that, have worse prognosis. Pancreatic cancer usually presents as a mass in the pancreas seen on CT. Pancreatic cancer is a very aggressive tumor with overall bad prognosis. Treatment requires a combination of chemotherapy, surgery and sometimes radiation therapy.
Neuroendocrine tumors of the pancreas
Neuroendocrine tumors of the pancreas originate from the endocrine cells of the pancreas, which produce hormones. The best-known hormone of the pancreas is insulin, but the insulin producing cells are not the most common to develop neuroendocrine tumors. The most common neuroendocrine tumors are in fact tumors that originate from endocrine cells but do not produce hormones at all. In that case they are called non-functional neuroendocrine tumors. They are also found on CT or MRI as masses in the pancreas. The best test for them is Gallium-DOTATETE scan or the older Octreotide-scan. They commonly spread to the liver. The treatment is surgical removal of the part of the pancreas and removal of the metastases to the liver. Neuroendocrine tumors are unique in a way that they are slower growing and usually less aggressive than conventional pancreatic cancer. This makes them uniquely positioned for surgical treatment and also portends better prognosis and longer survival for the patient. They are not treated with chemotherapy but may require hormonal treatment with octreotide.
Secondary malignant neoplasms of the pancreas
Even though not as often as the liver, occasionally the pancreas may be the site of spread from cancers originating in other organs. The following cancers are well known to spread to the pancreas:
Benign Diseases of the Pancreas
Benign diseases of the pancreas are not cancerous.
Pancreatitis
Pancreatitis is inflammation of the pancreas. It may be caused by many different factors, but the most common ones are gallstones and alcohol. More rare causes are medications, viruses, autoimmune, idiopathic. Idiopathic means that the cause is unknown. However, when there is a diagnosis of idiopathic pancreatitis, a thorough search for the possibility of underlying cancer has to be performed.
Acute pancreatitis
Acute pancreatitis may range from a mild disease which is only recognized by elevated pancreatic enzymes and abdominal symptoms to life-threatening devastating disease, requiring ICU, ventilator and blood pressure support, hemodialysis and multi-system organ failure. There is no surgical treatment for acute pancreatitis. If the cause is believed to be gallstones, the gallbladder needs to be removed on the same admission after the pancreatitis resolves. Surgery is the treatment for certain complications of acute pancreatitis. These include:
Necrotizing pancreatitis
Part of the pancreas dies as a result of acute pancreatitis and becomes a source of infection, that needs to be removed in order to control the infection. Usually the patients are very sick and are at risk of dying. Surgery is performed in an attempt to save their lives.
Pancreatic pseudocyst
Pancreatic pseudocyst is an abnormal cavity around the pancreas, filled with pancreatic juice. It looks like a cystic lesion but is not a cystic tumor but rather a walled-off fluid collection. It usually develops as a result of acute pancreatitis. Most commonly, it does not cause any problems and the pancreatic juice is absorbed naturally by the body and the cavity disappears. However, sometimes the fluid inside can get infected and then it requires drainage and antibiotics. Nowadays, the preferred way to drain it is endoscopically and that does not involve surgery. However, when the endoscopic drainage is not effective, then surgical drainage may be needed. That is usually done by connecting the cavity to the stomach or small bowel, which is called internal drainage. Drainage using tubes through the skin is not preferred but occasionally may need to be done if the patient is unstable.
Pancreatic fistula
Pancreatic fistula is a communication between the pancreatic duct and the skin. It may form as a result of acute pancreatitis, when the pancreatitis causes damage to the pancreatic duct, which leaks to a pseudocyst and the pseudocyst is drained through the skin. That is the main reason why drainage through the skin is not preferred for pseudocysts. Pancreatic fistula may also form as a result of surgery for necrotizing pancreatitis, when the dead pancreatic tissues is removed and that dead tissue involves the pancreatic duct. When there is a pancreatic fistula, the treatment initially is expectant – watch and wait approach – when there is an expectation that it will resolve on its own. To help with that an endoscopic pancreatic stent may be placed. If that expectant approach fails, then surgery may be needed. Surgery consists of removal of the part of the pancreas containing the disrupted pancreatic duct, causing the fistula.
Chronic pancreatitis
The textbook picture of chronic pancreatitis is defined by the 3D - Douleur (French - pain), Diarrhea and Diabetes. The pain is central and radiating to the back. The diarrhea and diabetes are due to insufficient production of pancreatic juice on one hand and insufficient production of insulin on the other hand by the damaged by chronic inflammation pancreas. There is no universal treatment. In selected patients an operation to create a new side-to-side connection between the pancreatic duct and the small bowel may be effective. In other patients, in whom a certain part of the pancreas is more affected than the rest, a removal of that part of the pancreas may be performed.
Pancreatic cystic lesions
Pancreatic cystic lesions are cystic tumors of the pancreas, filled with fluid. They have become more common in the modern era of wide use of CT and MRI because they are found more often and the question arises what to do with them. The main concern is always with the risk of these cysts transforming into cancer. The main reason why pancreatic cysts are of interest, is because we are trying to predict their likelihood in transforming into cancer. There are different types of pancreatic cysts and they have different risks of progressing to cancer. A key part of the workup of these cysts is a good quality “pancreatic protocol” CT or MRI and endoscopic ultrasound (EUS) with fine needle aspiration (FNA) of the cystic fluid for analysis. The fluid analysis allows us to divide the cysts in the following categories:
Serous Cysts of the Pancreas
Serous cysts of the pancreas are filled with clear fluid that resembles tears in appearance. They are common and they have essentially no risk to turn into cancer. If we can confidently confirm that a certain pancreatic cyst is serous, we can be certain that it has no risk of cancerous transformation. If there is no risk for cancerous transformation, then we do not have to follow such a cyst.
Mucinous Cysts of the Pancreas
Unlike the serous cysts of the pancreas, the mucinous cysts of the pancreas are filled with thicker fluid which may resemble mucus. Again, unlike serous cysts, mucinous cysts have a risk of malignant transformation. There are different types of mucinous cysts and their risk for cancer is different.
Mucinous cystic neoplasm
MCN is a mucinous cystic lesion of the pancreas which does not communicate with the pancreatic duct. It has a 10-50% risk of malignant transformation and because of that it is recommended for removal.
Intraductal mucinous cystic neoplasm (IPMN)
IPMN is a mucinous cystic lesion, which communicates with the pancreatic duct. There are different types of IPMN and they have different risks for cancerous transformation.
Main duct IPMN
Main duct IPMN means that the cyst involves the main pancreatic duct. It always requires surgical removal of the part of the pancreas involved.
Side branch IPMN
Side branch IPMN means that the cyst is involving a pancreatic duct which is a side branch of the main pancreatic duct. The malignant potential of side-branch IPMN is more difficult to estimate. Surgical removal is recommended if the cyst is greater than 3 cm and if it shows “worrisome features”. Worrisome features are wall thickening, wall enhancement on CT or mural nodules (areas of growth within the wall of the cyst). If these criteria are not present, then the cyst can be observed.
Solid pseuodpapillary tumor of the pancreas
This is a rare tumor of the pancreas which most commonly is benign, but it may have a more aggressive growth and it may contain an area of cancer. If preoperative diagnosis is made on endoscopic ultrasound (EUS) with fine needle aspiration biopsy (FNA), then surgery to remove the part of the pancreas involved is performed. If preoperative diagnosis is not made, then surgery to remove the part of the pancreas may be performed as an ultimate diagnostic test, which is also therapeutic.
Procedures on the Pancreas
Whipple procedure (pancreaticoduodenectomy)
Whipple procedure is a very complex operation, which is typically done for a cancer or other tumors of the head of the pancreas. It includes removal of the head of the pancreas, the duodenum, the lower part of the bile duct and the surrounding lymph nodes. The process requires working very close to multiple big vessels and each one of them is at risk of injury and massive bleeding. Once the above structures are removed, then connections (anastomoses) between the pancreas and the bowel, the bile duct and the bowel and the stomach and the bowel are made. These connections are at high risk of leaking of pancreatic juice, bile and stomach juice. The most common one is leaking of pancreatic juice, which is called a pancreatic leak or a pancreatic fistula. Pancreatic leak may vary in severity from a slight abnormality in the appearance or biochemical analysis of the drain fluid to a life-threatening condition, characterized by a large collection of infected fluid in the surgical bed, which may require placement of drainage tubes through the skin or repeat operation. Sometimes pancreatic leak may also be complicated by a late bleeding in the surgical bed, which also may be life-threatening. Another very common but less dangerous complication is delayed gastric emptying (DGE) also called gastroparesis. This may be a reason for prolonged inability to tolerate food by mouth, requiring TPN or tube feeding directly into the small bowel. The recovery from surgery, provided that there are no complications, is following the “rule of 6s” – 6 days in the hospital, 6 weeks to start eating normally and 6 months to start feeling back to normal. If there are complications, depending on their severity, the recovery would be delayed by days, weeks or months. Sometimes patients with severe complications may never recover back to normal. This is particularly important in the settings of cancer, because if the patient needs postoperative chemotherapy, the start of chemotherapy may be delayed significantly by surgical complications. In the worst case scenario, the patient would never be able to start chemotherapy, which would be otherwise indicated for the treatment of the cancer.
Distal pancreatectomy
Distal pancreatectomy is done for cancer or other tumors of the body and tail of the pancreas. When it is done for cancer it always includes removal of the spleen (splenectomy). The operation is done in two different ways depending on that if it is performed for cancer or for benign tumors, most commonly cysts. When it is done for cancer, it is called Radical Antegrade Modular Pancreatectomy and Splenectomy (RAMPS procedure), which removes all the tissue between the pancreas and the left renal vein with or without the left adrenal gland. When it is not done for cancer, then the operation is less radical and may be possible to preserve the spleen. When the spleen is planned for removal, the patient receives vaccines for Pneumococcus, Meningococcus and Hemophilus influenza type B (HiB). Similar to a Whipple procedure, distal pancreatectomy is associated with a risk of a leak of pancreatic juice. Similar to what is mentioned for Whipple procedure, pancreatic leak may vary in severity from a mild abnormality of the drain fluid to a life threatening condition, requiring multiple additional interventions.
Central pancreatectomy
Central pancreatectomy is an uncommonly performed procedures. It involves removal of a central part of the pancreas, usually neck-body. It results in two pancreatic stumps, one towards the head and one towards the tail. The stump towards the tail always requires a connection (anastomosis) to be made between the pancreatic duct and intestine (pancreatico-jejunostomy, similar to a Whipple procedure). The stump towards the head depending on the individual circumstances may require a second connection to be made, similar to the one at the tail, or to be left stapled across as a blind stump. Central pancreatectomy has double the risk of pancreatic leak as there are two pancreatic stumps. That is the reason why it is not commonly performed. At the same time, in special circumstances it can be used in order to preserve pancreatic tissue. This is a procedure which may be used in case of pancreatic cystic lesion, small neuroendocrine tumor, or metastatic disease, but would not be used for pancreatic cancer.
Puestow procedure
Puestow procedure is an operation that is done on patients with chronic pancreatitis, who have narrowing (stricture) of the pancreatic duct from scarring due to the chronic pancreatitis. Sometimes, the areas of narrowing are multiple and the duct has the appearance of a “chain-of-lakes”. Inability of the pancreatic juice to drain properly due to strictures may be responsible for the severe pain that patients with chronic pancreatitis experience. Puestow procedure filet-opens the pancreas along the length of the pancreatic duct and makes a long new connection with small bowel. That may result in improvement of pancreatic juice drainage and thus improvement of the pain. Because there is a connection (anastomosis) involving the pancreatic duct, there is a risk of pancreatic leak.
Debridement of pancreatic necrosis
Debridement of pancreatic necrosis is a procedure done for necrotizing pancreatitis. Pancreatic necrosis is the formation of area of dead pancreas as a result of severe acute pancreatitis, which is called necrotizing pancreatitis. The area of dead pancreas is a source of severe infection and severe inflammatory reaction, which may result in patient’s death. Thus it needs to be removed. Usually these patients are very sick and have multi-system organ failure. Debridement of pancreatic necrosis requires opening of the pancreas over the area where the dead pancreas is and pulling the dead tissue out. Usually there is a cavity filled with pus and, in the center, a portion of dead pancreas floats in the pus. The procedure may be associated with massive bleeding. It also may result in pancreatic fistula. Usually it is preferred to be performed through the stomach, which allows drainage of the residual cavity into the stomach, which is more effective than just putting drains in the debridement bed. When drained into the stomach, the risk of pancreatic fistula is also decreased.
Pancreatic cyst-gastrostomy
Pancreatic cyst-gastrostomy is a procedure that is done for pancreatic pseudocyst, which has an interface with the back wall of the stomach. First the front wall of the stomach is opened to get to the back wall. Then, the back wall is opened and through it the pancreatic pseudocyst is entered. The wall of the pseudocyst is sutured to the back wall of the stomach to allow a wide communication between the two. That facilitates an effective drainage of the pseudocyst into the stomach. Then the front wall of the stomach is sutured close. That is a form of internal drainage. Internal drainage means drainage into an intestine and not outside through the skin.
Pancreatic cyst-jejunostomy
Pancreatic cyst-jejunostomy is a procedure that is done for pancreatic pseudocyst, when it is not possible to perform an cyst-gastrostomy when there is no interface between the pseudocyst and the back wall of the stomach. In that case, the pseudocyst is opened from the front and a loop of intestine is brought to it and the two are sutured together to allow drainage of the pseudocyst into the intestine. That is a form of internal drainage.