Causes and predisposing factors:
- genetic predisposition. Often found in families with familial adenomatous polyposis. Also, in some patients, a cellular mutation of the K-ras gene is determined.
- BDS adenoma is a benign tumor of the papilla that can become malignant.
- Chronic diseases of the gallbladder and liver.
- Chronic pancreatitis.
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Symptoms and course of the disease
Cancer of the Vater papilla is detected in the early stages of development, due to the narrowing of the final section of the biliary tract. This leads to undulating yellowing with varying intensity of the skin, which is accompanied by itching. And refusal of food, indigestion, fever, vomiting leads to weight loss. Due to a violation of the outflow of bile, the liver enlarges, and the overflowing gallbladder can be felt through the abdominal wall. Obstruction of the excretory ducts is also displayed on the state of the blood.
The blood plasma shows:
- increased activity of gamma-glutamyl and alkaline phosphatase;
- significantly increased bilirubin;
- an increase in transaminase.
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Treatment of the disease
The only radical method of treatment - surgery. Most often, pancreatoduodenal resection is performed - removal of part of the duodenum, stomach and part of the pancreas with neighboring lymph nodes.
Of auxiliary importance are radiotherapy and chemotherapy. Chemotherapy is also used for metastases.
Palliative care is also performed. endoscopic intraductal interventions with a pronounced narrowing of the common bile duct, if it is impossible to perform a radical intervention. This type of operation includes papillotomy (dissection of the papilla of Vater) followed by stenting. This helps to normalize the passage of bile.
For effective treatment of cancer of the major duodenal papilla, high-quality early diagnosis is important.
Diagnosis of the disease
Diagnostic program:
- Consultation of a qualified specialist.
- Detailed blood tests, including general clinical, biochemical, electrolyte composition, lipid profile, determination of tumor markers, pancreatic enzymes, glycosylated hemoglobin.
- Ultrasound examination of the abdominal organs with dopplerography of the abdominal vessels; Spiral computed tomography of the abdominal cavity.
- Combined positron emission computed tomography.
- Endoscopic and laparoscopic ultrasonography.
- Esophagogastroduodenoscopy with Helicobacter pylori test (under anesthesia).
- Tumor biopsy.
- Urgent histopathology and histochemistry of biopsy material.
- Magnetic resonance cholangiopancreatography (as an option).
Prices
| Disease | Estimated price, $ |
|---|---|
| Prices for thyroid cancer screening | 3 850 - 5 740 |
| Prices for examination and treatment for testicular cancer | 3 730 - 39 940 |
| Prices for screening for stomach cancer | 5 730 |
| Prices for the diagnosis of esophageal cancer | 14 380 - 18 120 |
| Prices for the diagnosis and treatment of ovarian cancer | 5 270 - 5 570 |
| Prices for the diagnosis of cancer of the gastrointestinal tract | 4 700 - 6 200 |
| Prices for the diagnosis of breast cancer | 650 - 5 820 |
| Prices for the diagnosis and treatment of myeloid leukemia | 9 600 - 173 000 |
| Prices for the treatment of Vater's nipple cancer | 81 600 - 84 620 |
| Prices for rectal cancer treatment | 66 990 - 75 790 |
| Prices for the treatment of pancreatic cancer | 53 890 - 72 590 |
| Prices for the treatment of esophageal cancer | 61 010 - 81 010 |
| Prices for liver cancer treatment | 55 960 - 114 060 |
| Prices for the treatment of gallbladder cancer | 7 920 - 26 820 |
| Prices for stomach cancer treatment | 58 820 |
| Prices for the diagnosis and treatment of myelodysplastic syndrome | 9 250 - 29 450 |
| Prices for the treatment of leukemia | 271 400 - 324 000 |
| Thymoma treatment prices | 34 530 |
| Prices for lung cancer treatment | 35 600 - 39 700 |
| Prices for melanoma treatment | 32 620 - 57 620 |
| Prices for the treatment of basalioma | 7 700 - 8 800 |
| Prices for the treatment of malignant skin lesions | 4 420 - 5 420 |
| Prices for the treatment of melanoma of the eye | 8 000 |
| Prices for craniotomy | 43 490 - 44 090 |
| Prices for thyroid cancer treatment | 64 020 - 72 770 |
| Prices for the treatment of bone and soft tissue cancer | 61 340 - 72 590 |
| Prices for the treatment of laryngeal cancer | 6 170 - 77 000 |
| Prices for testicular cancer treatment | 15 410 |
| Prices for bladder cancer treatment | 21 280 - 59 930 |
| Prices for cervical cancer treatment | 12 650 - 26 610 |
| Prices for the treatment of uterine cancer | 27 550 - 29 110 |
| Prices for ovarian cancer treatment | 32 140 - 34 340 |
| Colon cancer treatment prices | 45 330 |
| Prices for the treatment of lymphoma | 11 650 - 135 860 |
| Prices for kidney cancer treatment | 28 720 - 32 720 |
| Prices for breast reconstruction after cancer treatment | 41 130 - 59 740 |
| Prices for breast cancer treatment | 26 860 - 28 900 |
| Prices for the treatment of prostate cancer | 23 490 - 66 010 |
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21625 0
Before the development and widespread introduction of endoscopic diagnostics, benign neoplasms in the OBD area were extremely rare. In recent years, due to the improvement of endoscopic equipment, benign tumors of BDS during endoscopy with biopsy are detected in 6.1-12.2% of cases. Benign tumors of BDS are equally common in both sexes, mainly in the middle age group.
More often, the development of papillomas in the BDS zone is observed with a separate confluence of the common bile duct and the pancreatic duct into the cavity of the duodenum (without the formation of a hepatic-pancreatic ampulla). It is believed that this anatomical structure contributes to the traumatization of the area of the mouths of the ducts during intestinal peristalsis, the development of congestive, inflammatory, fibrous and hyperplastic processes.
Pathomorphology
Macroscopically, with papillomatosis of the OBD, papillary growths are found at the mouth of the ducts of the papilla. Growths are small, bright pink or gray-red; they fill the opening of the ducts, protruding into the intestinal lumen. Papillomas have thin stalks associated with the mucosa of the OBD.Microscopically, papillomas consist of fibrous-epithelial and glandular (tubular glands) elements. Polyps are covered with high single-row prismatic epithelium, the cells of which have a light, weakly eosinophilic cytoplasm and a basally located nucleus. In the epithelium of papillomas, goblet cells and endocrinocytes are found. Often reveal areas of metaplasia (stratified squamous epithelium). Papillomas have a well-defined stroma containing vessels and cellular elements of connective tissue; often there are elements of chronic inflammation (lymphoplasmacytic infiltration).
BDS adenoma is also a benign epithelial tumor, but its prevalence in the population is slightly less than papillomatosis - 0.15%. According to foreign authors, based on autopsy materials, the frequency of detection of OBD adenomas ranges from 0.04-0.21%.
Macroscopically, the BDS adenoma is a single nodule or polypoid formation that fills the hepatic-pancreatic ampulla and protrudes into the lumen of the duodenum (adenoma prolapse). Tumor size within a few centimeters (usually 1-2 cm).
Microscopic examination reveals that the OBD adenoma is covered with an epithelium resembling the normal epithelium of the OBD and duodenum. The epithelium is similar to that of OBD papillomas, however, if cellular atypia is not expressed in papillomas, then there are features of atypia in the adenoma: cells and nuclei are large, the nuclei are hyperchromic, strongly elongated and located in the cells more chaotically than in papillomas, there are figures mitosis.
In addition, some cells do not secrete mucus while others hypersecrete. The stroma is weakly expressed. Adenomas in 12-23% of cases malignant (the same frequency with similar tumors of the colon); the risk is increased if the tumor is large or villous.
Sometimes in the lumen of the hepato-pancreatic ampulla or in the area of the distal common bile duct or pancreatic duct, hyperplastic intrapapillary polyps occur. Their development is associated with a productive component of chronic inflammation (papillitis). Macroscopically and microscopically, these polyps are identical to those with papillomatosis of the mouth of the ducts, the difference is only in the location.
A number of authors also include glandular-cystic hyperplasia of the transitional fold to benign formations of the OBD zone. Glandular cystic hyperplasia of the transitional fold is a fairly common pathology with the formation of cluster-like clusters in the OBD zone, which sometimes completely cover the mouth of the papilla, threatening the development of obstructive jaundice and pancreatitis. In most cases, this pathology is asymptomatic, and it is discovered by chance during EGDS.
Microscopically, this formation is represented by hyperplastic and cystic-dilated glands of the mucous membrane of the transitional fold of the duodenum.
During embryogenesis (heterotopy) transfer of hyperplastic papillary glands into the muscular structure of the BDS, papillary adenomyosis develops, a tumor-like proliferate of hyperplastic origin. This condition is characterized by hypertrophy of the muscle elements of the BDS. There are also assumptions about hormonal stimulation of these formations, like hyperplasia of the mammary or prostate gland.
Macroscopically, the OBD in adenomyosis has a spherical shape, reaching a diameter of 1.5 cm. The consistency of the papilla is dense, the mouth is almost impossible to determine. Microscopically, there are three forms (development phases) of BDS adenomyosis, which successively replace each other with the progression of the process:
. nodal;
. nodular diffuse;
. diffuse.
There are no mitoses, signs of destructive growth and cellular atypia in adenomyotic structures. This formation is attributed to neoplasms more because of the tumor-like macroscopic and clinical picture than in terms of morphological features.
Clinical picture
The manifestations of benign neoplasms of OBD are the same. In the early stages of the process, they depend not so much on the histological structure of the tumor, but on the degree of violation of the separation of bile and secretion of the pancreas, dysfunction of the sphincter of Oddi and motility of the duodenum. A characteristic picture of recurrent chronic cholecystitis, pancreatitis, secondary dysfunction of the sphincter of Oddi.Less commonly, the disease is manifested by recurrent mechanical jaundice, hepatic colic. Sometimes there are symptoms of chronic cholestasis in the form of prolonged skin itching, disorders of abdominal digestion in the duodenum and small intestine, and chronic constipation. Prolonged and increasing mechanical subhepatic cholestasis, characteristic of OBD cancer, is usually not present in benign neoplasms.
Diagnostics
Diagnosis of all benign neoplasms of BDS is based on the clinical picture, X-ray and endoscopic examination. Endoscopists have a rule: when examining the DP, always study the OBD zone. Differential diagnosis is carried out between papillomas and papillary cancer of the OBD. In any case, the diagnosis is specified according to the morphological study. Ultrasound, EUS, CT, MRI, MRCP, and ERCP are used to diagnose benign tumors of BDS, especially with large tumor sizes.Treatment
Surgical treatment. With papilloma yuse, EPST or endoscopic papillomectomy is performed. Small adenomas are usually removed endoscopically. For large tumors, papillotomy or papillomectomy with papilloplasty is performed, less often pancreatoduodenal resection. If malignancy is suspected, a pancreaticoduodenal resection is performed; in case of an inoperable process, a biliodigestive anastomosis is applied.Maev I.V., Kucheryavy Yu.A.
Pathology in the region of the major duodenal papilla (MDP) is of particular importance for the clinic, as it can quickly lead to impaired bile flow and require urgent measures aimed at its restoration.
The anatomical features of this area make it extremely vulnerable to pH changes, pressure drops, mechanical damage, detergent effects of bile and pancreatic juice. In this regard, papillitis is the most common pathology of OBD. Traumatization of the mucous membrane leads to stenosing papillitis, it may precede another pathology of OBD - a tumor lesion (benign and malignant).
benign tumors
Benign tumors of 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, neurofibromas. In some cases, adenoma may be complicated by malignancy.
Benign tumors of BDS can be asymptomatic for a long time and become an accidental finding during duodenoscopy. Histological examination of targeted biopsy materials allows clarifying the diagnosis. With preserved bile flow and the absence of 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%), weight loss (30%), anemia and diarrhea - in 5% of cases. The main method of diagnosis is endoscopy with targeted biopsy. CT is informative when the tumor size is more than 1 cm. Endoscopic ultrasopography is used to clarify the diagnosis.
In violation of bile flow and the presence of jaundice, surgical treatment is indicated. If the adenoma has a narrow base, then it can be removed endoscopically and the disturbed outflow of bile and pancreatic juice can be restored. If the tumor is located in the distal part of the papilla, amputation of the OBD is possible. If technical conditions allow, papillectomy is performed from endoscopic access. Due to the fact that papillectomy can lead to the closure of the mouth of the common bile duct, stents are placed in it and in the Wirsung duct, which are removed after a few days. If endoscopic adenomectomy fails, then they resort to surgical removal of the tumor - they excise the BDS and impose choledochoduodenoapastomosis. The same operation is performed in cases of suspected malignant degeneration of the tumor.
Malignant tumors
Cancer of the BDS can come 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, OBD cancer accounts for approximately 5% of all tumors of the gastrointestinal tract. There are no statistics on cholangiocellular cancer in Russia; according to hospital registries, OBD 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 ill (2:1), the average age of patients is 50 years.
F. Holzinger et al. 4 phases are distinguished in biliary carcinogenesis:
Phase II - genotoxic disorders leading to DNA damage and mutations;
Phase III - dysregulation of DNA reparative mechanisms and apoptosis, allowing mutated cells to survive:
Phase IV - further morphological evolution of premalignant cells into cholangiocarcinoma.
Pathological anatomy. Macroscopically, cancer of the OBD usually has a polypoid form, sometimes with an ulcerated tuberous surface, grows slowly and does not go beyond the OBD for a long time. Microscopically, the tumor is an adenocarcinoma, regardless of where it comes from. Adenocarcinomas emanating from the BDS ampulla have, like the right one, a papillary structure, they are distinguished by a low degree of malignancy, while aciparous cell carcinoma is characterized by infiltrative growth and rather quickly involves the surrounding tissues in the process. Metastases to regional lymph nodes appear when the tumor size is more than 2.5 cm, in about 25% of cases. Regional lymph nodes are affected first, followed by the liver and, less commonly, other organs. The tumor can invade the splenic and portal veins, cause their thrombosis and splenomegaly, and lead to a violation of the outflow of bile.
clinical picture. Often the first clinical manifestation is jaundice, slowly increasing, without a sharp deterioration in the general condition and pain attacks. On palpation, an enlarged gallbladder (Courvoisier's symptom) can be detected in 50-75% of cases of MDS cancer. Kypvoisier's symptom indicates distal obstruction of the biliary tract and is characteristic of both cancer of the obstructive duct and tumor of the head of the pancreas, and mechanical blockage of the distal common bile duct due to other causes.
At the same time, with a tumor with exophytic growth in the intestinal lumen, there may be no jaundice. 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 localization of the tumor, cholangitis occurs more often than with cancer of the pancreatic head (in 40-50% of cases). Infection of the pancreatic duct leads to pancreatitis.
The inflammatory component that has joined with OBD cancer can lead to serious diagnostic errors. Pain syndrome, fever, undulating jaundice give grounds for the diagnosis of cholecystitis, cholangitis, pancreatitis. After the use of antibiotics, inflammation is removed, the condition of some patients improves and they are discharged, mistakenly considering them recovered. Given 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 under conditions of hypotension makes it possible to suspect cancer of the duodenum - in the region of the Vater papilla, either a filling defect or a persistent and rough deformation of the wall, as well as a violation of the advancement of the contrast mass in this zone, is detected. Accurate topical diagnosis of OBD cancer with relaxation duodenography can be made in 64% of cases.
Duodenoscopy with targeted biopsy is the main method for diagnosing OBD cancer. In this case, the accuracy of targeted biopsy and the amount of biopsy material are important. With exophytic tumor growth, the information content of targeted biopsy ranges from 63 to 95%. ERCP may be performed to clarify the extent of the tumor. However, cannulation of the OBD 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 and the detection of cancer of the OBD is 58.8%.
Ultrasound diagnosis of OBD tumors is based on indirect symptoms, since they can rarely be visualized. An indirect sign of cancer is cholangioectasia along the whole length of the biliary tree, with blockade of the mouth of the Wirsung duct - pancreatectasia. OBD tumors and tumors emanating from the distal part of the common choledochus have a similar earographic picture and are practically indistinguishable from each other.
Ultrasound 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 allows final verification of OBD tumors.
Treatment. The main treatment for OBD cancer is surgery. It is considered the most curable tumor of the pancreatoduodenal zone, due to early diagnosis in 50-90% of cases the tumor is operable. Whipple's proximal duodenopancreatectomy is the method of choice. With cancer of the OBD, pancreatoduodenal resection 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.
Causes and predisposing factors:
- genetic predisposition. Often found in families with familial adenomatous polyposis. Also, in some patients, a cellular mutation of the K-ras gene is determined.
- BDS adenoma is a benign tumor of the papilla that can become malignant.
- Chronic diseases of the gallbladder and liver.
- Chronic pancreatitis.
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Symptoms and course of the disease
Cancer of the Vater papilla is detected in the early stages of development, due to the narrowing of the final section of the biliary tract. This leads to undulating yellowing with varying intensity of the skin, which is accompanied by itching. And refusal of food, indigestion, fever, vomiting leads to weight loss. Due to a violation of the outflow of bile, the liver enlarges, and the overflowing gallbladder can be felt through the abdominal wall. Obstruction of the excretory ducts is also displayed on the state of the blood.
The blood plasma shows:
- increased activity of gamma-glutamyl and alkaline phosphatase;
- significantly increased bilirubin;
- an increase in transaminase.
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Treatment of the disease
The only radical method of treatment - surgery. Most often, pancreatoduodenal resection is performed - removal of part of the duodenum, stomach and part of the pancreas with neighboring lymph nodes.
Of auxiliary importance are radiotherapy and chemotherapy. Chemotherapy is also used for metastases.
Palliative care is also performed. endoscopic intraductal interventions with a pronounced narrowing of the common bile duct, if it is impossible to perform a radical intervention. This type of operation includes papillotomy (dissection of the papilla of Vater) followed by stenting. This helps to normalize the passage of bile.
For effective treatment of cancer of the major duodenal papilla, high-quality early diagnosis is important.
Diagnosis of the disease
Diagnostic program:
- Consultation of a qualified specialist.
- Detailed blood tests, including general clinical, biochemical, electrolyte composition, lipid profile, determination of tumor markers, pancreatic enzymes, glycosylated hemoglobin.
- Ultrasound examination of the abdominal organs with dopplerography of the abdominal vessels; Spiral computed tomography of the abdominal cavity.
- Combined positron emission computed tomography.
- Endoscopic and laparoscopic ultrasonography.
- Esophagogastroduodenoscopy with Helicobacter pylori test (under anesthesia).
- Tumor biopsy.
- Urgent histopathology and histochemistry of biopsy material.
- Magnetic resonance cholangiopancreatography (as an option).
Prices
| Disease | Estimated price, $ |
|---|---|
| Prices for thyroid cancer screening | 3 850 - 5 740 |
| Prices for examination and treatment for testicular cancer | 3 730 - 39 940 |
| Prices for screening for stomach cancer | 5 730 |
| Prices for the diagnosis of esophageal cancer | 14 380 - 18 120 |
| Prices for the diagnosis and treatment of ovarian cancer | 5 270 - 5 570 |
| Prices for the diagnosis of cancer of the gastrointestinal tract | 4 700 - 6 200 |
| Prices for the diagnosis of breast cancer | 650 - 5 820 |
| Prices for the diagnosis and treatment of myeloid leukemia | 9 600 - 173 000 |
| Prices for the treatment of Vater's nipple cancer | 81 600 - 84 620 |
| Prices for rectal cancer treatment | 66 990 - 75 790 |
| Prices for the treatment of pancreatic cancer | 53 890 - 72 590 |
| Prices for the treatment of esophageal cancer | 61 010 - 81 010 |
| Prices for liver cancer treatment | 55 960 - 114 060 |
| Prices for the treatment of gallbladder cancer | 7 920 - 26 820 |
| Prices for stomach cancer treatment | 58 820 |
| Prices for the diagnosis and treatment of myelodysplastic syndrome | 9 250 - 29 450 |
| Prices for the treatment of leukemia | 271 400 - 324 000 |
| Thymoma treatment prices | 34 530 |
| Prices for lung cancer treatment | 35 600 - 39 700 |
| Prices for melanoma treatment | 32 620 - 57 620 |
| Prices for the treatment of basalioma | 7 700 - 8 800 |
| Prices for the treatment of malignant skin lesions | 4 420 - 5 420 |
| Prices for the treatment of melanoma of the eye | 8 000 |
| Prices for craniotomy | 43 490 - 44 090 |
| Prices for thyroid cancer treatment | 64 020 - 72 770 |
| Prices for the treatment of bone and soft tissue cancer | 61 340 - 72 590 |
| Prices for the treatment of laryngeal cancer | 6 170 - 77 000 |
| Prices for testicular cancer treatment | 15 410 |
| Prices for bladder cancer treatment | 21 280 - 59 930 |
| Prices for cervical cancer treatment | 12 650 - 26 610 |
| Prices for the treatment of uterine cancer | 27 550 - 29 110 |
| Prices for ovarian cancer treatment | 32 140 - 34 340 |
| Colon cancer treatment prices | 45 330 |
| Prices for the treatment of lymphoma | 11 650 - 135 860 |
| Prices for kidney cancer treatment | 28 720 - 32 720 |
| Prices for breast reconstruction after cancer treatment | 41 130 - 59 740 |
| Prices for breast cancer treatment | 26 860 - 28 900 |
| Prices for the treatment of prostate cancer | 23 490 - 66 010 |
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Medicines are prescribed with caution in patients who have been exposed to high doses of ionizing radiation to the pelvic area or who have previously taken alkylating agents.
Contraindications to chemotherapy for cancer of the major duodenal papilla:
- hypersensitivity to the components of the drug;
- extreme degree of exhaustion;
- bone marrow failure syndrome;
- severe systemic infections or the threat of their occurrence;
- kidney failure;
- abnormally low levels of protein in the blood plasma;
- pregnancy and lactation (refusal to feed for the period of treatment);
- poor blood clotting;
- progressive decrease in the number of platelets;
- hearing impairment.
Medications can be canceled with a pronounced hypertensive reaction to the introduction of cytotoxins (changes in heart rate and increased blood pressure).
Etiology and pathogenesis
More often, the development of papillomas in the BDS zone is observed with a separate confluence of the common bile duct and the pancreatic duct into the cavity of the duodenum (without the formation of a hepatic-pancreatic ampulla). It is believed that this anatomical structure contributes to the traumatization of the area of the mouths of the ducts during intestinal peristalsis, the development of congestive, inflammatory, fibrous and hyperplastic processes.
Preparation
Preparation, or premedication, for chemotherapy for cancer of the major duodenal papilla takes place beforehand as an independent diagnostic procedure. The patient is examined for the functioning of the liver and kidneys, digestive organs and hematopoiesis. His psychosomatic status and the work of the central nervous system are revealed.
Carefully examine the general and biochemical composition of blood for bilirubin, the level of amylase enzymes, creatinine, glucose, and total protein. When risky conditions are identified, complex drug support with a compensatory effect is prescribed.
To improve the tolerance of chemotherapy for cancer of the major duodenal papilla, a diet low in fat and carcinogens and increased hydration may be recommended.
Classification and pathomorphology
Macroscopically, with papillomatosis of the OBD, papillary growths are found at the mouth of the ducts of the papilla. Growths are small, bright pink or gray-red; they fill the opening of the ducts, protruding into the intestinal lumen. Papillomas have thin stalks associated with the mucosa of the OBD.
Microscopically, papillomas consist of fibrous-epithelial and glandular (tubular glands) elements. Polyps are covered with high single-row prismatic epithelium, the cells of which have a light, weakly eosinophilic cytoplasm and a basally located nucleus.
In the epithelium of papillomas, goblet cells and endocrinocytes are found. Often reveal areas of metaplasia (stratified squamous epithelium). Papillomas have a well-defined stroma containing vessels and cellular elements of connective tissue; often there are elements of chronic inflammation (lymphoplasmacytic infiltration).
BDS adenoma is also a benign epithelial tumor, but its prevalence in the population is somewhat less than papillomatosis - 0.15%. According to foreign authors, based on autopsy materials, the frequency of detection of OBD adenomas ranges from 0.04-0.21%.
Macroscopically, the BDS adenoma is a single nodule or polypoid formation that fills the hepatic-pancreatic ampulla and protrudes into the lumen of the duodenum (adenoma prolapse). The size of the tumor is within a few centimeters (usually 1-2 cm).
Microscopic examination reveals that the OBD adenoma is covered with an epithelium resembling the normal epithelium of the OBD and duodenum. The epithelium is similar to that of OBD papillomas, however, if cellular atypia is not expressed in papillomas, then there are features of atypia in the adenoma: cells and nuclei are large, the nuclei are hyperchromic, strongly elongated and located in the cells more chaotically than in papillomas, there are figures mitosis.
In addition, some cells do not secrete mucus while others hypersecrete. The stroma is weakly expressed. Adenomas in 12-23% of cases become malignant (the same frequency with similar tumors of the colon); the risk is increased if the tumor is large or villous.
Sometimes in the lumen of the hepato-pancreatic ampulla or in the area of the distal common bile duct or pancreatic duct, hyperplastic intrapapillary polyps occur. Their development is associated with a productive component of chronic inflammation (papillitis).
A number of authors also include glandular-cystic hyperplasia of the transitional fold to benign formations of the OBD zone. Glandular-cystic hyperplasia of the transitional fold is a fairly common pathology with the formation of cluster-like clusters in the OBD zone, which sometimes completely cover the mouth of the papilla, threatening the development of obstructive jaundice and pancreatitis.
Microscopically, this formation is represented by hyperplastic and cystic-dilated glands of the mucous membrane of the transitional fold of the duodenum.
When hyperplastic papillary glands move in embryogenesis (heterotopy) into the muscular structure of the BDS, papillary adenomyosis develops - a tumor-like proliferate of hyperplastic origin. This condition is characterized by hypertrophy of the muscle elements of the BDS.
Macroscopically, the OBD in adenomyosis has a spherical shape, reaching a diameter of 1.5 cm. The consistency of the papilla is dense, the mouth is almost impossible to determine. Microscopically, there are three forms (development phases) of BDS adenomyosis, which successively replace each other with the progression of the process:
nodal;
nodular diffuse;
diffuse.
There are no mitoses, signs of destructive growth and cellular atypia in adenomyotic structures. This formation is attributed to neoplasms more because of the tumor-like macroscopic and clinical picture than in terms of morphological features.
The classification of malignant tumors of OBD according to the TNM system is as follows.
T1 - the size of the tumor does not exceed 1 cm, the tumor extends beyond the papilla.
T2 - a tumor no larger than 2 cm, involved in the process of the mouth of the common bile duct and pancreatic duct, but without infiltration of the posterior wall of the duodenum.
T3 - tumor up to 3 cm, sprouts the posterior wall of the duodenum, but without germination in the pancreas.
T4 - the tumor spreads beyond the duodenum, grows into the head of the pancreas, captures the vessels.
Ny - the presence of lymphogenous metastases is not known.
Na - single retroduodenal lymph nodes are affected.
Nb - parapancreatic lymph nodes are affected.
Ne - affected periportal, para-aortic or mesenteric lymph nodes.
M0 - no distant metastases.
M1 - distant metastases are present.
There are several morphological types of malignant tumors of OBD.
Adenocarcinoma BDS.
papillary cancer. Characterized by exophytic growth in the lumen of the papilla and duodenum. The tumor is represented by glandular-like complexes of small size with a well-defined stroma. The complexes are cavities lined with high columnar epithelium with a thickened basement membrane.
Scirrhous form. The tumor is small in size with a predominant spread along the common bile duct and into the surrounding tissues. The neoplasm contains fibrous tissue rich in collagen fibers with a pronounced vascular network, among which small cancerous polymorphic cells are visible, sometimes forming cavities and cysts;
Mucous cancer. Growth into the lumen of the papilla of glandular structures formed by prismatic cells with a large amount of pink mucus in the apical regions is characteristic. The mitotic activity of cancer cells is high.
Adenocarcinoma originating from duodenal epithelium. A large number of glandular structures of a round, oval or twisted shape are revealed, devoid of excretory ducts and in some places overflowing with mucus. These structures infiltrate the submucosal and muscular layers of the duodenum.
Of all the listed malignant neoplasms of the OBD zone, adenocarcinoma most often develops. OBD carcinomas are characterized by slower growth and a more favorable prognosis than pancreatic cancer.
Three forms of OBD cancer are macroscopically distinguished: polypous, infiltrative and ulcerative. Usually the tumor is small (up to 1.5 cm in diameter) and has a stalk. The process does not go beyond the papilla for a long time.
The polypous form can lead to obstruction of the lumen of the obstructive junction (see Fig. 5-45), and the infiltrative form can lead to its stenosis. In addition, the tumor can infiltrate the wall of the duodenum with the formation of a nodular form. This form of tumor is characterized by the absence of changes in the mucous membrane above the tumor, so a superficial biopsy may not give results.
The infiltration of the BDS by the tumor process goes through the submucosa and muscular membranes of the papilla, and then through the wall of the common bile duct, pancreatic tissue, and duodenal wall. Usually, metastases to the peripancreatic lymph nodes occur when the tumor diameter is more than 15 mm.
A long-term tumor process is characterized by increasing cholestasis, secondary cholecystitis, development of congestive gallbladder, choledocholithiasis, cholangitis, secondary biliary hepatitis, liver cirrhosis, biliary-dependent obstructive pancreatitis.
Damage to the duodenum by a tumor process can lead to its pronounced deformation, the development of secondary dynamic and mechanical obstruction (duodenostasis), and ulceration to bleeding. Clinical picture
Cancer of the OBD area can occur in the form of several clinical forms:
choleic-like variant (with typical biliary colic);
cholangitic (without colic, with skin itching, jaundice, subfebrile condition);
gastric (diskinstic) with secondary gastric dyspepsia.
Once having arisen, jaundice in OBD cancer becomes permanent with a tendency to worsen, however, temporary (false) improvements are possible], mainly due to recanalization of the duct during tumor decay, or against the background of anti-inflammatory therapy due to a decrease in secondary mucosal edema.
A pronounced dyspeptic syndrome is characteristic, associated with a violation of abdominal digestion in the duodenum and small intestine due to a violation of the outflow of bile and pancreatic secretions. Gradually, patients lose weight, up to cachexia.
How is chemotherapy done for cancer of the major duodenal papilla?
Medicines are administered intravenously by bolus or drip method. The dosage is selected individually, taking into account body weight, maximum daily dose and age of the patient. The active substance is dissolved in sterilized saline.
Infusions are carried out in a course mode until the appearance of primary side effects (diarrhea, stomatitis, a drop in the level of leukocytes and platelets), after which the treatment is suspended. After normalization of indicators, the cycle is repeated.
In some cases, round-the-clock administration of the dosage form in a sparing dosage is acceptable.
Chemotherapy for adenoma of the major duodenal papilla is highly toxic, systemically affects the work of all life-supporting functions and can cause a number of negative disorders in the functioning of organs:
- inflammation of the mucous membrane of the oral cavity, esophagus, rectum and bladder;
- nausea, vomiting;
- diarrhea;
- decrease in the number of erythrocytes, leukocytes and platelets per unit volume of blood;
- motor disorder and impaired coordination of movements;
- angina;
- myocardial diseases;
- body hyperpigmentation;
- confusion;
- disorientation in space;
- allergic reactions of anaphylactic properties.
Cytotoxics cause reversible alopecia, hearing loss and altered taste sensations, and may lead to skin deterioration and nail loss.
Prices for a course of chemotherapy depend on the nature of the disease and the cost of the method (combined or monotherapy) of treatment in Moscow clinics for cancer of the major duodenal papilla, the composition of the drugs, a set of diagnostic procedures and the rehabilitation period.
The pricing takes into account the status of the medical institution, the qualifications of the medical staff, the profile of the hospital and the level of accommodation.
Clinical picture
The manifestations of benign neoplasms of OBD are the same. In the early stages of the process, they depend not so much on the histological structure of the tumor, but on the degree of violation of the separation of bile and secretion of the pancreas, dysfunction of the sphincter of Oddi and motility of the duodenum.
Less commonly, the disease is manifested by recurrent mechanical jaundice, hepatic colic. Sometimes there are symptoms of chronic cholestasis in the form of prolonged skin itching, disorders of abdominal digestion in the duodenum and small intestine, and chronic constipation.
Diagnostics
Diagnosis of all benign neoplasms of BDS is based on the clinical picture, X-ray and endoscopic examination. Endoscopists have a rule: when examining the DP, always study the OBD zone.
Differential diagnosis is carried out between papillomas and papillary cancer of the OBD. In any case, the diagnosis is specified according to the morphological study. Ultrasound, EUS, CT, MRI, MRCP, and ERCP are used to diagnose benign tumors of BDS, especially with large tumor sizes.
Diagnosis is carried out taking into account clinical signs, more often the syndrome of obstructive jaundice, X-ray and endoscopic examination data with biopsy. However, the stage of the process can often be determined only during the operation (metastases are found in the lymphatic tract and surrounding organs, more often in the head of the pancreas).
Radiographically, in case of malignant neoplasms of the OBD, a defect in the filling of the duodenum in the zone of its descending part along the internal contour is detected. The size of the defect, as a rule, is small (up to 3 cm), its contours are uneven, the mucosal relief is disturbed.
Particular attention should be paid to the rigidity of the intestinal wall at the site of the filling defect. Diagnosis is helped by tight filling of the intestine with barium sulfate in conditions of hypotension, as well as double contrasting of the intestine.
The most common early endoscopic symptom is an increase in the size of the OBD, ulceration in its area, papillary or tuberous formations (see Fig. 5-46). Often the papilla acquires a crimson-red color.
Particular attention during endoscopy should be given to examining the condition of the longitudinal fold of the duodenum. In cancer of the OBD, a bulging of its oral region is often detected, without gross violations of the relief of the mucosa, which is characteristic of the infiltrating growth of the OBD tumor and the presence of biliary hypertension.
In some cases, ERCP, MRCP, and EUS help diagnose cancer with OBD; these methods make it possible to identify damage to the ducts., the transition of the process to the pancreas.
In case of unsuccessful attempts to contrast the ducts due to tumor obstruction of the mouth of the BDS, laparoscopic or percutaneous transhepatic cholecystocholangiography is used. As a rule, dilatation of the bile ducts is detected with a “breakage” of the common bile duct in the duodenum.
Differential diagnosis in the presence of obstructive jaundice syndrome is carried out with benign tumors of obstructive pulmonary disease, choledocholithiasis, stenosing papillitis, pancreatic head tumors, autoimmune pancreatitis, etc.
With extensive tumor infiltration and ulceration of the OBD area, secondary damage to the papilla most often occurs due to the spread of cancer of the pancreatic head. The correct diagnosis can be made by CT, MRI, ERCP, ultrasound due to the detection of changes in the structure of the gland, indicating its primary tumor lesion.
Diagnosis is associated with significant difficulties due to non-specific symptoms. In the process of diagnosis, the oncologist focuses on complaints, data from an objective examination, radiography, transhepatic or intravenous cholangiography, duodenal sounding, fibrogastroduodenoscopy and other studies.
A sufficiently reliable study is duodenal sounding, during which it is often possible to detect blood in the duodenal contents. Sometimes during this study, neoplasia cells and pancreatic enzymes are detected.
Radiographic signs of cancer of the major duodenal papilla are uneven contours or filling defect in the area of the inner wall of the duodenum, as well as the lack of patency or deformation of the bile duct in the area close to the Vater nipple.
When conducting fibrogastroduodenoscopy, a tumor-like formation is detected and an endoscopic biopsy of the suspicious area is performed. In some cases, the diagnosis of cancer of the major duodenal papilla cannot be established using standard methods; to clarify the nature of the pathology, it is necessary to perform a laparotomy, dissect the nipple veils, take tissue, and then decide on the extent of the operation based on the data of an urgent histological examination.
Treatment
Surgical treatment. With papilloma yuse, EPST or endoscopic papillomectomy is performed. Small adenomas are usually removed endoscopically. For large tumors, papillotomy or papillomectomy with papilloplasty is performed, less often pancreatoduodenal resection.
Maev I.V., Kucheryavy Yu.A.
Treatment is surgical, including radical surgery, despite the high operational risk.
The prognosis for untimely surgery is unfavorable.
"Cancer of the major duodenal papilla, symptoms, diagnosis, prognosis" - Diseases of the liver and biliary tract
For small tumors in the early stages, transduodenal papillectomy is usually used with the imposition of a bypass biliodigestive anastomosis. The five-year survival rate for this operation is 9-51%. You can perform an extended papilectomy according to N.N. Blokhin or pancreatoduodenal resection.
With advanced tumor processes, operations are more often performed to drain the ducts of the BDS (EPST, the imposition of various cholecystodigestive anastomoses). At the same time, timely radical surgical treatment ensures a five-year survival rate of 40%.
For palliative purposes in patients with inoperable MDS cancer, due to low trauma and the possibility of re-execution in case of relapses of obstructive jaundice, the use of EPST with retrograde prosthetics (stenting) of the bile ducts is indicated.
These data indicate the importance of timely diagnosis of tumor lesions in the OBD zone: the earlier the tumor process is verified, the more radical and less traumatic it is possible to operate on these patients.
The main method of treating this pathology is surgery, which, depending on the prevalence of the process, can be radical or palliative. The group of palliative operations includes about ten different types of anastomoses, which allow to restore the outflow of bile into the digestive tract or (less often) to prevent compression of the duodenum by the growing cancer of the major duodenal papilla.
Radical surgery is a difficult and difficult intervention, therefore, it is carried out only after careful selection of patients in accordance with standards, including an acceptable degree of malnutrition, blood protein levels, certain indicators of pulse and lung capacity, etc.
Patients with cancer of the major duodenal papilla undergo gastropancreatoduodenal resection. If there are contraindications to radical intervention, conditionally radical operations are performed: papillectomy, duodenectomy or economical pancreatoduodenal resection. Radiotherapy and chemotherapy for cancer of the major duodenal papilla are ineffective.