Thymoma (thymus Tumor) Treatment
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Thymoma is a tumor, originating from epithelial tissue of thymus gland. Thymoma is considered to be very rare. It may manifest itself in different ways. For instance, symptoms of thymus cancer may vary from asymptomatic to severe, when person feels compression in the chest, pain and intoxication. Thymoma is a tumor of mediastinal organ, which is a central compartment of chest. In 65-70% of thymoma cases disease has a benign course. Growth with invasion of pleura and pericardium is seen in 30% of patients and distant metastases happen in 5%. Thymoma is more common in women 40-60 years of age. The reasons for the formation of thymus gland tumor are unknown. It is assumed that thymoma can have fetal origin and it is caused by disruptions of certain hormonal synthesis, thymopoietin. Factors which can cause growth and development of tumor are infectious diseases, radiation exposure, and injuries of mediastinal organ. It is noticed that the thymoma is often combined with a variety of other endocrine and autoimmune diseases. In oncology there are three types of thymus cancer: benign, malignant type 1 and malignant type 2.
Benign thymoma, which accounts for 50-70% of this disease has a diameter less than 5 cm. Malignant thymoma occurs in 9-20% of cases. Often it has a pronounced lymphocytic infiltration. Prognosis for both benign and malignant thymus cancer is satisfactory, if treatment was started on time. 20-year survival rate in this case is more than 90%.
Thymus gland is located at the bottom of the neck in the anterior upper mediastinum and it secretes such hormones, as thymulin and b-thymosin. Thymus gland is formed during the first month of child'sl ife. Children who have thymoma usually have thymic hyperplasia, which may even lead to the development of mediastinal compression syndrome.
There are two types of thymoma: lymphoid and epithelial thymoma. Lymphoid thymoma is less aggressive compared to epithelial one. Thymoma staging includes:
- Stage I - tumor is encapsulated and not big in its size
- Stage II - invasion of pleura capsule by malignant cells
- Stage ||| - invasion of mediastinal structures (pericardium, blood vessels, and heart)
- Stage IVa - dissemination of the tumor to pleura and pericardium
- Stage IVb - lymphatic and hematogenous metastasis
Clinical manifestations and course of thymoma depend on type of person`s hormonal activity and size of tumor. More than half of thymoma tumor cases develop latently and are detected during routine radiographic examination. Invasive thymoma cause mediastinal compression syndrome, which is accompanied by such thymoma symptoms as:
- Pain in the chest
- Dry cough
- Shortness of breath
- Compression of trachea
- Cyanosis (blue coloration of skin)
- Respiratory failure
- Puffiness of face
- Swelling of the neck veins
Pressure on the nerve nodes causes development of Horner's syndrome, hoarseness and elevation of the diaphragm dome. Compression of esophagus is accompanied by swallowing disorder. Patients with progressive thymoma cancer report:
- Decrease in muscle strength
- Fatigue
- Weakness of facial muscles
- Double vision
- Choking
- Voice disorder
In severe cases, they may develop myasthenic crisis and then patient are required transition to mechanical ventilation and tube feeding.
In late staging cancer shows signs of intoxication, which includes fever, anorexia and weight loss. Thymoma is also often accompanied by a variety of hematologic and immunodeficiency symptoms.
Pain observed in benign and malignant thymoma is usually moderate and is felt in lesion, but it can spread to interscapular region, shoulder and neck. Pain often occurs on the left side and can be manifested in angina. If there is pain in bones, there may be metastases. If there are metastases in patient`s body there can also be problems with sweating, dilated pupils, physical changes in the affected area of the body, increased temperature.
In case of damage of laryngeal nerve endings, there is hoarseness. It becomes difficult for a patient to speak for long periods of time. If phrenic nerve was damaged, diaphragm can change its position. Also large venous trunks become damaged because of compression syndrome. Manifestations of this syndrome include disruptions in venous outflow of blood from the upper body to head.
Patients with thymoma may experience noise in the head, because of swelling of the veins in chest and neck. Swelling of veins also results in shortness of breath. Compression of trachea causes appearance of cough.
Diagnosis of thymoma is held by a group of specialists, which includes oncologists, radiologists, endocrinologists, and neurologists. Clinical examination can identify expansion of chest veins bulging belly and increase in cervical and supraclavicular lymph nodes. Also signs of compression syndrome and myasthenia gravis can be detected during such general examination.
Radiological methods of patient`s examination of neoplasm in thymus include fluoroscopy, radiography and tomography of the chest. Contrast study of esophagus can also be performed. Chest radiograph of thymoma diagnosis any malformations of irregular shape, located in anterior mediastinum. CT of the chest greatly increases the amount of information obtained by primary X-ray diagnostics. Asymptomatic tumor of the thymus is more difficult to detect, because its parameters are very little. Tumors less than 3 cm in diameter can be identified by computer tomography. Nevertheless, main method of thymoma diagnosis is X-ray. An integrated X-ray examination in most cases determines localization of the pathological process. Tumor can be developing in mediastinum or its surrounding tissues and organs (such as lungs, diaphragm, and chest). The X-ray can give a rounded shadow, if a patient has thymoma, but the most characteristic thymoma sign is pear-shaped shadow with sharp end pointing downwards. Shadows may be located in the middle of X-Ray image, but very often they are found on one side. Rarely thymoma is localized at the bottom. If boundaries and location of thymoma can not be traced, it is impossible to understand the size and shape of tumor mass. In that case only tomography can give a detailed study of pneumomediastinum. Tomography allows seeing changes in shape and shade of formation. Very often thymoma can be mistaken for cystic formation. Lobed pattern indicates the presence of thymoma.
The differential diagnosis is carried out with retrosternal goiter of the thyroid gland, tumors of the sternum, as well as malignant tumors originating from the fiber and mediastinal lymph nodes.
Fluoroscopy makes it possible to detect a pathological shadow and give information about its location, shape, size, mobility, intensity and general contours. Fluoroscopy can also indicate presence or absence of walls` pulsation. Fluoroscopy usually verifies data obtained by X-ray. If there are enlarged lymph nodes in supraclavicular areas a doctor can perform biopsy, which allows to determine if thymus tumor is benign or malignant. Patients with thymoma suspicion also need to submit general and biochemical blood test.
- Treatment of thymic tumors usually involves surgical intervention. Surgical removal of the thymus gland is the main treatment method for this condition. In benign thymoma neoplastic formation is removed along with thymus gland, fatty tissue, and lymph nodes of mediastinum .Thymus glands and lymph nodes must be removed, if there is high risk of tumor recurrence.
- During radiation therapy doctors use high-energy x-rays to completely destroy or diminish the number of cancer cells. Side effects of radiation therapy include: weakness, skin reactions, swallowing disorder, indigestion, and constipation. In late stages of thymoma, doctors can also prescribe several courses of chemotherapy. Radiosurgery is the most effective method for dealing with tumors located in remote locations and with dimensions greater than 20 mm. It is also applied if a person has brain metastases.
- The most modern means of radiosurgery is a robotic complex, called cyberknife. Radiosurgical treatment with cyberknife can reach tumor with great precision at 1400 sheaves of high power ionizing. Such ionizing radiation destroys malignant cells much faster than traditional radio frequency. At the same time, healthy tissue around tumor remains intact. The biggest disadvantage of radiofrequency treatment is that it can irradiate not only malignant tissues, but also healthy cells, which are vital for a health of a person. That`s why radiofrequency has so many side effects, which mostly are responsible for the damage of immune system that a person has during cancer treatment. Nevertheless, modern radiofrequency technologies help patients with thymoma avoid such side effects. Cyberknife is one of such technologies. It is especially good for thymoma treatment as it can destroy malignant cells, which could not be reached during surgery. Surgery with cyberknife is carried out by unique computer guidance system, which eliminated human factor and is the most precise surgery option, which is available in medical world today. The process of treatment in this case is completely painless. There is no need for rigid fixation of patient on the table, as cyberknife responds to slightest movements of patient`s body, automatically correcting the direction of radiation beam. Treatment does not require more than 5 sessions (fractions) for 30-40 minutes each. There were many precautions before this type of treatment was introduced, because it was not used enough. Nowadays cyberknife treatment for eliminating tumor is used in many hospitals, although such treatment can be expensive.
- Chemotherapy in thymoma also aims to destroy cancerous, malignant cells by stopping their activity and growth. Chemotherapy is also prescribed by oncologist. Chemotherapy thymoma treatment is sometimes performed before surgery to shrink the size of thymus cancer. It is also required if a patient has stage four. Doses and number of chemotherapy courses is determined by thymoma stage and patient`s individual susceptibility to certain drugs. Some patients do not respond to certain chemotherapy drugs, thus they need medicines which are stronger. Chemotherapy in thymoma can be difficult for a surgeon to detect a whole amount of tumor size, and it may be dangerous if he leaves any malignant cells behind, as they have tendency to grow. The side effects also include weakness, nausea, hair loss, considerable loss of appetite, and sometimes diarrhea. These side effects usually vanish once treatment is finished and a patient can resume his normal lifestyle.
- Another option for thymoma treatment is targeted therapy. Targeted therapy destroys specific genes or proteins that can provoke the growth of new cancerous cells. For thymoma anti-angiogenesis therapy can be a good option, as it stops angiogenesis. Generally, angiogenesis is responsible for creating new blood vessels. Cancerous cells are in need of special nutrients that are produced by blood vessels, that`s why tumorous cells die if angiogenesis has stopped. If a person is in his last stage of thymoma and it is impossible to conduct surgery, he may need to undergo palliative therapy, which is also known as supportive care. It increases the quality of patient`s life and gives him all support he needs. For instance, many patients with cancer tend to be depressed because of their condition and constant weakness which does not stop even after a good night sleep. They need emotional and psychological help from professionals who can guide them through this difficult period of their life. Palliative care mostly focuses on alleviating the symptoms and easing side effects which could arise after radiofrequency or chemotherapy. Palliative treatment also uses special relaxation techniques which can provide patient with emotional support. Other therapies are also at discretion of palliative care specialists.
Authors: Dr. Nadezhda Ivanisova, Dr. Farrukh Ahmed
