Internal Medicine

Optic disc carcinoma

definition

Optic disc carcinoma is a relatively rare, malignant tumor of the disc. As a papilla (Major duodenal papilla or Papilla Vateri) one denotes the mouth of the common duct of the main bile duct (Common bile duct) and pancreatic duct (Pancreatic duct) in the duodenum (Duodenum). The tumor leads to constriction of the papilla and the resulting digestive disorders.

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causes

Optic disc carcinomas are extremely rare tumor diseases that mainly occur in young patients between the ages of 20 and 30. These are adenocarcinomas, which are malignant tumors that arise from the glandular tissue of the gastrointestinal tract. In the case of papillary carcinoma, cell changes lead to an increase in the glandular tissue in the area of the papilla vateri.

Cell changes are caused by mutations that cause the cell to grow in an uncontrolled manner, no longer die and can no longer differentiate into a certain type of tissue.

A distinction is made between benign (Papillary adenoma) and malignant growths (Optic disc carcinoma). In the course of the disease, however, a benign papillary adenoma often develops into a malignant papillary carcinoma. It is therefore advisable to have even benign growths completely removed by a surgical procedure in order to keep the risk of degeneration low.

Familial adenomatous polyposis (FAP) is a risk factor for the development of papillary tumors. This is a congenital disease in which those affected develop numerous polyps (mucosal protuberances) in the large intestine due to a genetic mutation (see also: Colon polyps).

Smoking, the use of oral contraceptives (“birth control pills”) and parasitic infections that infest the liver (eg liver fluke infections) are further risk factors. In addition, as with many other cancers, a genetic predisposition, environmental influences and lifestyle are likely to play an important role Role.

diagnosis

In most cases, optic disc cancer is caused by accompanying symptoms, such as jaundice (Jaundice) or inflammation of the pancreas (acute pancreatitis) diagnosed. The doctor will take an ultrasound scan of the abdomen (abdomen), in which the backflow of the bile fluid and the enlarged biliary tract are noticeable.

The duodenum (Duodenum), the biliary tract and the pancreatic duct can also be visualized from the inside using endoscopic retrograde cholangiopancreatography (ERCP). The final diagnosis of disc carcinoma is made after a tissue sample has been taken (biopsy) with subsequent tissue examination by a pathologist. Under the microscope, typical changes in the cell morphology and disturbances in the tissue architecture can be seen, which suggest a malignant tumor.

Tumor markers

The tumor markers are determined as part of a blood test. Elevated values of the markers CA19-9 and CEA indicate a malignant tumor of the papilla, but are in themselves insufficient for the diagnosis and therefore have no diagnostic significance. The increase in these tumor markers can also have other, harmless causes. An imaging procedure (ERCP, MRI, CT) and a biopsy must be performed for a clear diagnosis of disc carcinoma.

Concomitant symptoms

Optic disc carcinomas often remain symptom-free for a long time and can grow unnoticed. Only when the tumor has reached a certain size does mechanical displacement of the papilla occur in the area of the duodenum, which results in symptoms. Common complaints are disorders of the bile drainage and inflammation of the pancreas.

Accompanying symptoms of a papillary tumor are pain in the upper abdomen and colicky cramps. The patients complain of a feeling of pressure and abdominal pain. The narrowing of the biliary tract (obstructive cholestasis) the tumor can cause jaundice (post-hepatic jaundice) train. Jaundice can be easily diagnosed by typical symptoms, such as yellowing of the skin and sclera (the white part of the eyes).

The bile can no longer get into the intestines through the tumor and patients suffer from digestive disorders, diarrhea and colorless stools. As a result, there is weight loss and a decrease in appetite. Other unspecific symptoms can be sudden fever, fatigue and a pronounced feeling of weakness. During a blood test, the doctor can determine anemia and high levels of inflammation, usually caused by inflammation of the pancreas (acute pancreatitis).

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metastasis

If the papillary carcinoma forms daughter tumors (Metastases) the prognosis is significantly worse. The tumor then affects the lymph nodes in the area, especially along the arterial vessels. The tumor cells can spread through the body via the lymph vessels and form metastases.

therapy

Surgical removal of the tumor is the treatment of choice for papillary carcinoma. The tumor tissue on the papilla is cut out with a certain safety margin to the surrounding healthy tissue (Excision), partial removal of the pancreas and duodenum may also be necessary. In the case of larger carcinomas, the entire head part of the pancreas including the gallbladder and the adjacent duodenum is removed (Whipple pancreatoduodenectomy).

To be on the safe side, the lymph nodes that supply the affected area are also removed. A surgical technique that allows the stomach to be preserved is usually chosen. Since important parts of the digestive system were removed anyway, patients must receive nutritional therapy after the operation to avoid complications.

Among other things, the meals must be taken in several small portions throughout the day and should be rather low in fat. The digestive enzymes missing from the removed pancreas must also be taken in tablet form.

A stent can be inserted into the draining bile or pancreatic ducts in patients in whom the papillary carcinoma is already very advanced and who can therefore no longer be operated on. The stent allows the digestive secretion to flow away unhindered and the jaundice can be eliminated.

chemotherapy

Chemotherapy slows down the growth of disc cancer, but does not bring a permanent cure. In addition to the sometimes very strong side effects, chemotherapy also has only a low response rate in papillary carcinoma and is therefore only used for palliative purposes.

Prognosis - What is the life expectancy with optic disc cancer?

The prognosis and thus the life expectancy of a papillary carcinoma depend on the size of the tumor and the tumor stage. The tumor stage is determined using the so-called TNM classification and describes the type of tumor, its extent and whether lymph nodes are involved.

The 5-year survival rate after successful surgical removal of the carcinoma is between 40 and 85%, depending on the exact location. If metastases have already formed in other parts of the body, the prognosis is worse.

What are the chances of a cure with optic disc carcinoma?

In most cases, optic disc carcinomas make themselves felt through the early symptoms (painless jaundice, acute pancreatitis). Surgical removal of the tumor tissue can lead to a good chance of recovery. After successful removal of the optic disc carcinoma, the relapse rate is (Recurrence rate) relatively low and the majority of patients are considered cured.