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In the structure of the incidence of malignant neoplasms, cancer of the major duodenal papilla accounts for about 1%. There are no gender differences in incidence. Risk factors that can lead to the development of cancer include the presence of hyperplastic changes in the area of the papilla of Vater - hyperplastic orifice polyps, adenomas, glandular-cystic hyperplasia of the transitional fold of the major duodenal papilla, adenomyosis.
Major duodenal papilla cancer most often presented as an exophytic form that bleeds easily on instrumental palpation. The tumor has the appearance of a polyp, papilloma or fungal growth, sometimes a “cauliflower” appearance. The obstructive jaundice that develops may be remitting in nature. Rarer endophytic forms of cancer cause persistent jaundice. The macro- and microscopically defined boundaries of the tumor in cancer of the major duodenal papilla coincide much more often than in exocrine pancreatic cancer or cancer of the common bile duct. In tumor tissue, individual and grouped endocrine cells of a tumor nature are often identified, having a cylindrical, triangular and spindle-shaped shape. Such cells are found in the greatest numbers in highly differentiated tumors - papillary and tubular adenocarcinomas. As anaplasia increases, the frequency of detection of endocrine cells decreases until they are completely absent.
Cancer of the major duodenal papilla has a pronounced infiltrating growth: already by the time jaundice appears, there may be invasion of the wall of the duodenum, pancreas, metastases in regional, juxtaregional lymph nodes and distant metastases. In most cases, the tumor grows into the wall of the common bile duct and completely obstructs its lumen. But obstruction or stenosis may be incomplete - disturbances in the neuromuscular apparatus of the duct and swelling of the mucous membrane are quite enough to significantly reduce or completely stop the flow of bile into the duodenum. Biliary hypertension develops, in which all overlying parts of the biliary tree undergo dilatation. There is a real threat of cholangitis and cholangiogenic liver abscesses. In the liver itself, the mechanisms of its cirrhotic transformation are launched. Hypertension in the pancreatic ducts, caused by stenosis or obstruction of the main pancreatic duct by a pancreatic tumor, leads to degenerative-dystrophic and inflammatory changes in the pancreatic parenchyma. An increase in tumor size can lead to deformation of the duodenum. In this case, obstruction of the intestinal lumen by a tumor, as a rule, does not lead to decompensation of intestinal patency. A more common complication after obstructive jaundice is tumor disintegration with intraintestinal bleeding.
The size of the tumor during the period of obstructive jaundice syndrome and surgical treatment is from 0.3 cm. The paths of lymphogenous metastasis are the same as for cancer of the head of the pancreas and the common bile duct. The frequency of detection of metastases in regional and juxtaregional lymph nodes in LBD cancer at the time of surgery is 21-51%. Characteristically, one or two groups of lymph nodes of the regional collector are affected.
The close location of the large duodenal papilla to the pancreatic and bile ducts makes it very vulnerable in the event of the development of a pathological process in the large pancreatic and common bile ducts, as well as in the duodenum. Regular changes in pressure in this area of the duodenum additionally have a traumatic effect on the papilla.
For this reason, there is a relatively mild development of chronic and acute duodenal papillitis. With chronic papillitis, benign and, in some cases, malignant neoplasms of the BDS occur. The concept of the major duodenal papilla includes the papilla itself, the terminal section of the common bile duct and the ampulla of the papilla.
Major duodenal papilla carcinoma is an epithelial malignant tumor, initially arising from the epithelium of the duodenal mucosa, which covers the papilla and adjacent areas of the intestine, the epithelium of the pancreatic duct, the epithelium of the ampulla of the ampulla, and the acinar cells of the pancreas, which is adjacent to the region of the major duodenal papilla.
It is often very difficult to determine the place where the tumor began to develop. Basically, carcinoma appears as a medullary tumor or polyp. Carcinoma of acinar origin often acquires infiltrative growth. Regarding the structure, the most common are adenocarcinomas. Carcinoma, which originates from the epithelium of the ampulla of the major duodenal papilla, is distinguished by its papillary structure and relatively low malignancy. Its size, as a rule, does not exceed 3 centimeters.
The first symptom of the disease is often obstructive or subhepatic jaundice, which manifests itself as a result of compression of the common bile duct. Basically, jaundice develops gradually, painlessly and without a sudden disturbance in the general condition. Often, when first seeing a patient, a doctor makes an erroneous diagnosis - viral hepatitis.
Subhepatic jaundice, especially in the initial period, is incomplete. This stage is characterized by the appearance of stercobilin in the feces and urobilin in the urine, as well as slight skin itching, in comparison with carcinoma of the head of the pancreas and cholangiocarcinomas.
Occasionally, pain in the upper half of the abdomen can be observed in the early stages. 1-3 months before jaundice, the patient begins to lose weight. Significant weight loss is observed already with the appearance of jaundice. Further progression of the disease is sometimes accompanied by the development of purulent cholangitis. More common symptoms are bleeding from the tumor and compression of the duodenum.
In addition, there is an increase in aminotransferase activity and a significant increase in GGTP activity. A small proportion of patients experience an increase in leukocytes and an increase in ESR.
X-ray examination of the duodenum of patients helps to identify a picture that raises suspicion of a tumor of the papilla of Vater: the corresponding zone has a filling defect, or a gross and persistent deformation of any wall. Usually, various disturbances in the advancement of the contrast mass in the area of the nipple are always detected.
Duodenal endoscopy can also provide valuable diagnostic data. During endoscopy, a biopsy is performed on areas suspicious for the presence of a tumor. If any doubts arise or a specialist wishes to clarify the area of tumor spread, ERCP can also be used. However, it is not always possible to cannulate the papilla.
During radionuclide scintigraphy, there is often a delay in the flow of bile into the duodenum; CT and ultrasound, which are performed for the first time, often do not provide significant diagnostic information. The most aggressive course of the tumor process can be observed with the atomic origin of the neoplasm. The rate of progression of this type of tumor is similar to that of the ductal type. The ampullary type is less aggressive. In addition, it can be detected the fastest because it causes jaundice to begin relatively earlier. The most slowly progressing type is considered duodenal.
If this is possible, then pancreatoduodenal resection is performed. Palliative operations involving the application of biliodigestive anastomoses and biliary prostheses have become quite widespread. In case of duodenal stenosis, gastroenterostomosis is applied. If necessary, chemotherapy is given.
As with other tumors, the fate of the patient depends on the time of detection of the tumor.
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Pathology in the area of the major duodenal papilla (MDP) is of particular importance for the clinic, as it can quickly lead to disruption of bile outflow and require urgent measures aimed at its restoration.
The anatomical features of this area make it extremely vulnerable to changes in pH, pressure changes, mechanical damage, and the detergent effects of bile and pancreatic juice. In this regard, papillitis is the most common pathology of BDS. Trauma to the mucous membrane leads to stenotic papillitis; it can precede another pathology of BDS - a tumor lesion (benign and malignant).
Benign tumors
Benign tumors of the BDS are very rare - in 0.04-0.1% of cases - and are more often represented by adenomas (villous and tubular). Less common are lipomas, fibromas, leiomyomas, and neurofibromas. In some cases, adenoma may be complicated by malignancy.
Benign tumors of the abdominal cavity can be asymptomatic for a long time and become an accidental finding during duodenoscopy. Histological examination of targeted biopsy materials allows us to clarify the diagnosis. If bile outflow is preserved and there are no clinical manifestations, dynamic endoscopic observation is indicated.
Clinical manifestations are characterized by jaundice in 70% of cases, dull or colicky pain in the right hypochondrium (60%), loss of body weight (30%), anemia and diarrhea in 5% of cases. The main diagnostic method is endoscopy with targeted biopsy. KT turns out to be informative when the tumor size is more than 1 cm. Endoscopic ultrasopography is used to clarify the diagnosis.
If bile flow is impaired and jaundice is present, surgical treatment is indicated. If the adenoma has a narrow base, then it can be removed endoscopically and the impaired outflow of bile and pancreatic juice can be restored. If the tumor is located in the distal part of the papilla, amputation of the BJ is possible. If technical conditions allow, papillectomy is performed from endoscopic access. Due to the fact that papillectomy can lead to closure of the mouth of the common bile duct, stents are installed in it and in the Wirsung duct, which are removed after a few days. If endoscopic adenomectomy cannot be performed, then they resort to surgical removal of the tumor - the BDS is excised and choledochoduodenoapastomosis is applied. The same operation is also performed if malignant degeneration of the tumor is suspected.
Malignant tumors
BDS cancer can originate from the epithelium of the duodenal mucosa covering the papilla of Vater, directly from the ampulla of the BDS, the epithelium of the pancreatic duct and the acinar cells of the pancreas adjacent to the duct. According to the literature, BDS cancer accounts for approximately 5% of all gastrointestinal tract tumors. In Russia, there are no statistics on cholangiocellular cancer; according to hospital registries, BDS cancer accounts for 7-8% of malignant neoplasms of the periampullary zone. According to foreign statistics, the incidence of biliary tumors varies from 2 to 8 per 100,000 inhabitants.
Risk factors include smoking, diabetes mellitus, and a history of gastric resection. Men are more often affected (2:1), the average age of patients is 50 years.
F. Holzinger et al. There are 4 phases in biliary carcinogenesis:
Phase II - genotoxic disorders leading to DNA damage and mutations;
Phase III - dysregulation of DNA repair mechanisms and apoptosis, allowing mutated cells to survive:
Phase IV - further morphological evolution of premalignant cells into cholangiocarcinoma.
Pathological anatomy. Macroscopically, BDS cancer usually has a polypoid shape, sometimes with an ulcerated, tuberous surface, grows slowly and does not extend beyond the BDS for a long time. Microscopically, the tumor is an adenocarcinoma, regardless of where it comes from. Adenocarcinomas emanating from the ampulla of the ampulla, like the right one, have a papillary structure and are characterized by a low degree of malignancy, while aciparnocellular carcinoma is characterized by infiltrative growth and quite quickly involves surrounding tissues in the process. Metastases to regional lymph nodes appear when the tumor size is more than 2.5 cm, in approximately 25% of cases. The regional lymph nodes are the first to be affected, then the liver and, less commonly, other organs. The tumor can invade the splenic and portal veins, cause thrombosis and splenomegaly, and lead to disruption of the outflow of bile.
Clinical picture. Often the first clinical manifestation is jaundice, which slowly increases, without a sharp deterioration in the general condition and pain attacks. Upon palpation, an enlarged gallbladder (Courvoisier's symptom) can be detected in 50-75% of cases of BDS cancer. Kypvoisier's symptom indicates distal obstruction of the bile ducts and is characteristic of both BDS cancer and a tumor of the head of the pancreas, as well as mechanical block of the distal common bile duct due to other reasons.
At the same time, with a tumor with exophytic growth into the intestinal lumen, jaundice may not occur. However, the tumor ulcerates early and may be complicated by bleeding. Ulceration of the tumor contributes to its infection and penetration of the infection into the bile ducts with ascending cholangitis. With this tumor localization, cholangitis occurs more often than with cancer of the head of the pancreas (in 40-50% of cases). Infection of the pancreatic duct leads to pancreatitis.
The inflammatory component associated with BDS cancer can lead to serious diagnostic errors. Pain, fever, and wavy jaundice provide grounds for diagnosing cholecystitis, cholangitis, and pancreatitis. After the use of antibiotics, inflammation is relieved, the condition of some patients improves and they are discharged, mistakenly considered to have recovered. Considering the high prevalence of biliary pathology and cholelithiasis, in particular cholelithiasis, it is impossible to narrow the search for the causes of jaundice. The combination of BDS cancer with cholelithiasis and cholecystitis is 14%.
Diagnostics. X-ray examination of the duodenum in conditions of hypotension allows one to suspect cancer of the duodenum - in the area of the papilla of Vater, either a filling defect or a persistent and gross deformation of the wall is detected, as well as a violation of the advancement of the contrast mass in this area. An accurate topical diagnosis of BDS cancer can be made with relaxation duodenography in 64% of cases.
Duodenoscopy with targeted biopsy is the main method for diagnosing BDS cancer. In this case, the accuracy of the targeted biopsy and the amount of biopsy material are important. With exophytic tumor growth, the informative value of targeted biopsy ranges from 63 to 95%. To clarify the area of tumor spread, ERCP can be performed. However, cannulation of the BDS is successful in 76.5% of cases. Failures are due to the impossibility of introducing a contrast agent into the bile and pancreatic ducts due to their blockade by the tumor. If necessary, the study is supplemented with percutaneous transhepatic cholangiography. The information content of the method in detecting BDS cancer is 58.8%.
Ultrasound diagnosis of BDS tumors is based on indirect symptoms, since they are rarely visualized. An indirect sign of cancer is cholangiectasia along the entire length of the bile tree, and with blockage of the mouth of the Wirsung duct - pancreatectasia. OBD tumors and tumors arising from the distal part of the common bile duct have a similar uchographic picture and are practically indistinguishable from each other.
Ultrasound examination and laparoscopy help to differentiate acute surgical diseases of the hepatobiliary region and conditions caused by damage to the major duodenal papilla. Duodenoscopy with biopsy makes it possible to definitively verify tumors of the abdominal cavity.
Treatment. The main treatment method for BDS cancer is surgery. It is considered the most curable tumor of the pancreaticoduodenal zone; thanks to early diagnosis, in 50-90% of cases the tumor is operable. The method of choice is proximal duodenopancreatectomy according to Whipple. For BDS cancer, pancreaticoduodenectomy is performed. Transduodenal local extirpation of the duodenal papilla is a palliative intervention. With partial duodenopancreatectomy, mortality does not exceed 10%, with extirpation of the duodenal papilla - less than 5%. At stage I, the 5-year survival rate is 76%, at stages II and III - 17%. In general, the 5-year survival rate of patients after surgery is 40-60%.
Due to the rarity of this form of cancer, oncologists do not have much experience with chemotherapy.
The papilla of Vater (also known as the major duodenal papilla) is located in the duodenum. This is the anastomosis of the common bile and pancreatic ducts. Cancer of this papilla is the third most common cause of obstructive jaundice.
Cancer of the papilla of Vater develops due to the transformation of the cells of the pancreatic or bile duct, next to which it is located, or the cells of the epithelium of the duodenum. The tumor grows slowly. The pathological anatomy is as follows: visually the neoplasm resembles cauliflower inflorescences or papilloma, may have the shape of a mushroom, and in rare cases endophytic forms are observed. The tumor quickly ulcerates; at the time of removal, a diameter of 3 mm is most often recorded.
For cancer of the duodenal papilla (major duodenal papilla), it is common to invade the bile flow. The affected area is the walls of the duodenum and the pancreas. There is a threat (21–51%) of the appearance of lymphogenous metastases. Distant metastases can develop in the liver, adrenal gland, lungs, bones, and brain, but this occurs in rare cases.
The growth of a BDS tumor into the intestinal wall can cause bleeding, leading to anemia. Upon palpation, the patient can clearly feel the enlarged gallbladder under the liver.
At the moment, scientists find it difficult to accurately name the causes of the development of a tumor of the papilla of Vater, but some risk factors have been identified.
Men suffer from the disease more often (2:1). Carcinoma usually appears around the age of 50. Working in hazardous chemical production increases the risk of developing the disease.
The first symptom is obstructive jaundice due to narrowing of the bile duct. Initially, it moves and becomes more stable as the disease progresses. During this phase, symptoms such as severe pain, profuse sweating, chills and itching are also observed.
In most cases, cancer of the major duodenal papilla leads to sudden weight loss and vitamin deficiency. Indicators may also include symptoms such as digestive disorders: bloating, pain, diarrhea (stool is gray in color). If the disease is advanced, fatty stool may appear.
The development of metastases can change the nature of pain. Affected organs become depleted and function poorly.
Diagnosis of a malignant tumor of BDS is often difficult due to the similarity of symptoms of various diseases. For example, stenotic duodenal papillitis (BD stenosis) may have a number of similar symptoms, in particular the development of jaundice. Adenoma of the intestinal tract also leads to the proliferation of intestinal tissue.
Diagnosis is complicated by inflammatory processes associated with cancer. Often, such symptoms provide grounds for diagnosing pancreatitis, cholecystitis, etc. After a course of antibiotics, the inflammation is relieved, which is mistakenly perceived as recovery. Inflammation can also occur due to papillitis of the BDS.
In addition, the complex anatomy of the papilla of Vater often makes the diagnosis difficult. To make an accurate diagnosis, data obtained from an objective examination, duodenoscopy, cholangiography (intravenous or transhepatic), probing and other studies are usually used.
The main diagnostic method is duodenoscopy with targeted biopsy. If the tumor grows exophytically, it is clearly visible (the accuracy of the study is 63–95%). Failures are possible due to stricture of the ducts, due to which the contrast agent does not spread well.
An X-ray examination of the duodenum is often used. In the presence of a tumor of the abdominal cavity, disturbances in the movement of the contrast agent are visualized and changes in the anatomical shape of the walls or filling of the intestine become clearly visible. This method is also used to diagnose duodenal papillitis.
In some cases, when the BDS is not reliably visualized and standard examinations do not allow an accurate diagnosis, this means the need for laparotomy - the nipple is cut to collect tissue.
In some cases, endoscopy or gastroscopy of the stomach with examination of the gastrointestinal tract is used.
Treatment must be prompt. The main method is surgical intervention. The patient undergoes gastropancreatoduodenal resection. This type of treatment is difficult for the body and is allowed for patients after checking their level of exhaustion, the amount of protein in the blood and other indicators.
If cancer treatment begins at stage I or II, the survival rate is 80–90%. At stage III, it also makes sense to start treatment: the five-year life expectancy in this case reaches 5–10%.
If the patient's health condition does not allow radical therapy, treatment consists of conditionally radical operations, for example pancreaticoduodenectomy.
If there is no hope for the patient's recovery, palliative therapy is used, which is aimed at alleviating symptoms. In particular, they ensure the outflow of bile using various types of anastomoses. Such treatment not only alleviates suffering, but in some cases can prolong the patient’s life.
Chemical therapy in this case is practically ineffective.
It is difficult to overestimate the importance of proper nutrition. It should be taken into account that the condition of BDS is adversely affected by both overeating and abuse of junk food (smoked, fried, etc.), as well as malnutrition, in particular grueling diets or fasting, which are carried out at your own discretion without consulting a doctor. If you have gastrointestinal diseases (duodenitis, cholecystitis, etc.), you must strictly follow the prescribed diet.
Frequent stress and chronic fatigue should also be avoided.
In this video, a specialist will talk about the disease of the papilla of Vater and the difficulties of diagnosing the disease.
Causes and predisposing factors:
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Cancer of the papilla of Vater is detected in the early stages of development, due to narrowing of the final section of the biliary tract. This leads to wavy yellowing of varying intensity of the skin, which is accompanied by itching. And refusal to eat, indigestion, fever, vomiting leads to weight loss. Due to a violation of the outflow of bile, the liver becomes enlarged, and a full gallbladder can be palpated through the abdominal wall. Obstruction of the excretory ducts is also reflected in the state of the blood.
In blood plasma it is noted:
The only radical treatment method is surgery. Most often, pancreatoduodenal resection is performed - removal of part of the duodenum, stomach and part of the pancreas with adjacent lymph nodes.
They have auxiliary value radiation therapy and chemotherapy. Chemotherapy is also used for metastases.
Palliative measures are also performed endoscopic intraductal interventions with pronounced narrowing of the common bile duct, if it is impossible to perform radical intervention. This type of operation includes papillotomy (dissection of the papilla of Vater) followed by stenting. This helps normalize the passage of bile.
For effective treatment of cancer of the major duodenal papilla, high-quality early diagnosis is important.
Diagnostic program:
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