Mediastinal tumors 1. Briefly describe the topographic anatomy of the mediastinu m.

The mediastinum is limited laterally by the two pleural cavities, superiorly by the bony structures of the narrow thoracic and inferiorly by the diaphragm. A nd classically described in three divisions: 1 - anterior mediastinum - limited by the sternum, the thoracic aperture, the diaphragm and pericardium and posteri orly by the bodies of the first four thoracic vertebrae. Has the thymus, aortic arch and its branches! innominate veins and lymphatic tissue and areolar. 2 - Me diastinum average - and limited superiorly by the pericardial sac and extends to the fourth thoracic vertebra. Contains the heart and pericardium. the trachea, hilum of each lung and tracheobronchial lymph nodes. 3 - Mediastinum later - lie s between the pericardium and the vertebral column and extends superiorly to the anterior mediastinum. It contains the esophagus, vagus nerve, sympathetic nerve chain, thoracic duct, descending aorta, azygos vein system and hemi-azygos, and paravertebral lymph areolar tissue. 2. Classification and incidence of primary lesions of the mediastinum. - Neurogenic tumors, thymomas, and congenital cysts are most common and account 6O% of the lesions. Other disorders are often found localized mediastinal lymphomas (13%) and germ cell tumors (11%). The relative f requency of specific mediastinal tumors is different in adult and pediatric pati ents. Neurogenic tumors are more prevalent in children under four years, while d uring adolescence the incidence of lymphoma is greatly increased. Of congenital cysts. bronchogenic and enteric cysts are diagnosed at all ages. while pericardi al cysts are uncommon in children. Moreover, thymomas and endocrine tumors repre sent rare in childhood. - The primary tumors and cysts of the mediastinum repres ent lesions that generally must be treated by surgical means but there are many other disease processes that manifest with mediastinal masses secondary Extendin g substernal goiter neck. aneurysms of the aorta and its major branches. metasta tic tumors. systemic granulomas. diaphragmatic hernias. esophageal carcinomas. p aravertebral abscesses, and the meningocele many other disorders must be differentiated from primary tumors and cysts. 3. Ma jor signs and symptoms associated with tumors of the mediastinum. Most series re port 56-65% of symptomatic patients and the presence of symptoms correlates well with the histologic type of mediastinal lesions. Symptomatic mediastinal masses are malignant 50%, while the masses were asymptomatic, 90% are benign. The most frequent complaints are chest pain, cough, dyspnea, recurrent respiratory infec tions and dysphagia, which is usually due to compression exerted by the results of injury or invasion of adjacent mediastinal structures. Less frequently, there is obstruction of the vena cava superior vocal cord paralysis, Horner's syndrom e or spinal cord compression. An interesting group of patients have primary medi astinal tumors or cysts causing pressure on the heart or great vessels, prevent cardiac performance and produce symptoms that mimic primary heart disease. 4. Sy mptoms associated with systemic syndromes secondary to endocrine function of med iastinal tumors. Carcinóde Cushing's disease, thymoma Gynecomastia germ cell t umor Hypertension, Pheochromocytoma, ganglioneuroma, chemodectomas Diarrhea Ganglioneuroma Hypercalcemia of parathyroid adenoma, lymphoma, intrathoracic goiter Thyrotoxicosis Hypoglycemia mesothelioma, teratoma, fibrosarcoma, ne urossarcoma 5. Systemic syndromes associated with primary mediastinal tumors and cysts. - Thymoma: myasthenia gravis, pure aplasia of erythrocytes, hypogammaglo bulinemia, Whipple's disease, megaesophagus. collagen vascular disease. malignan cy, myocarditis. - Carcinoid, thymoma: multiple endocrine adenomatosis. - Neurofibroma, neurilemmoma, mesothelioma: osteoarthropathy. - Enteric Cysts: v ertebral anomalies. - Lymphoma: fever of unknown origin. - Hodgkin's disease: pa in induced by alcohol. - Neuriblastoma: opsomioclonia. 6. Most common sites of m ediastinal masses. - Mediastinum previous: thymoma, teratoma, seminoma, lymphoma , carcinoma, parathyroid adenoma, intrathoracic goiter, lipoma, lymphangioma, ao rtic aneurysm. - Mediastinum average: Lymphoma. chaste pericardial bronchogenic cyst, metastatic tumor, systemic granuloma. - Mediastinum later: neurogenic tumo r, bronchogenic cyst, enteric cyst, xanthogranuloma, diaphragmatic hernia, menin gocele,€paravertebral abscess. 7. What are the diagnostic potential of being use

d for clarification of mediastinal masses? - Chest radiography (PA and P): Perha ps the most useful diagnostic procedure, because it shows the size, density, pre sence of calcification and the anatomical location of you. - Tomografla biplane or front: You can make the differentiation between mediastinal masses and normal structures, which is useful in evaluating vertebral deformities or backpack att ached. - Fluoroscopy: Can be used to differentiate a lesion of the mediastinum a djacent pulmonary parenchyma. - X-ray with barium ingestion: To demonstrate inva sion, extrinsic compression or displacement of the esophagus. - Angiocardiografl a and Thoracic Aortography: Held in selected patients for the differentiation of vascular lesions of nãovasculares. - Myelography: To document the extent of int ramedullary tumors or subsequent to differentiate a neurogenic tu a meningocele. - Pneumomediastinografia: Held mostly in children to differentiate between thymo ma and thymic hyperplasia. - Computerized Axial Tomography: Provides accurate cr oss-sectional images of anatomical areas previously viewed the RX. This is a rev iew of high precision to define almost all of mediastinal masses. - Mapping with radioisotopes is extremely useful in the evaluation of mediastinal masses earli er for the presence of ectopic thyroid tissue. - Transthoracic fine needle aspir ation: low-risk procedure to obtain samples for histological diagnosis. The draw back is sometimes the lack of material for examination - Exploration of the ante rior mediastinum and mediastinoscopy: Represent alternative procedures for biops y of lesions that are not eligible for surgical resection. 8. What are the main types of neurogenic tumors of the mediastinum? - Neurilemmoma - And thou most co mmon, arises from the neural sheath, occurs in the third and fifth decades and a ppears as a homogeneous density the RX. When there is mucoid degeneration and he morrhage can be observed cyst formation and calcification. Can grow into the int ervertebral slab even in the absence of clinical evidence of extension into the spinal column. - Neurofibroma - Contains elements as well as nerve cells of the nerve sheath. Unlike neurilemmomas these lesions are ma! encapsulated. May repre sent a manifestation of generalized neurofibromatosis, however if a patient has von Recklinghausen's disease, is most likely a posterior mediastinal mass is a m eningocele. - Neurossarcoma - malignant degeneration may occur as the neurilemmo ma and neurofibroma. A. Advanced age and neurofibromatosis increase the risk of this possibility, which is of poor prognosis. - Ganglioneuroma - to originate fr om sympathetic ganglia and are more common in children. It is composed of mature ganglion cells and nerve fibers. Are well encapsulated and reach a considerable size before causing symptoms. It is a benign lesion. - Ganglioneuroblastoma - It consists of immature ganglion cells and can metastas ize widely. - Neuroblastoma - And thou SN friendly more undifferentiated. He pre sents as small cells in rosettes, 10% of which appear as primary mediastinal les ions. Children usually have a fever, anorexia, cough and diarrhea. At the time o f diagnosis in most cases there are already metastases, brain, liver and regiona l lymph nodes. - Pheochromocytoma - is rare in the mediastinum. is clinically in distinguishable from an abdominal injury. For this you vascularization is often visualized by thoracic aortography. - Paraganglioma or chemodectomas - originate from existing chemoreceptor tissue around the aortic arch and the vagus nerve. The recent evidence confirms that these tumors may secrete catecholamines such a s pheochromocytomas. Although histologically appear benign and only rarely produ ce metastasis, the spread of aggressive local you can produce significant morbid ity and mortality. * The benign you should be surgically excised. When the intra medullary extension, it must be excised before or simultaneously with thoracotom y. If surgical resection is incomplete, radiotherapy 9. Clinical features, diagn osis and treatment of thymoma. Clinical features and diagnosis is the most commo n cancer in the anterior mediastinum. Their recognition has increased by more ag gressive surgical approach in patients of myasthenia gravis pruners. Occur with equal frequency in men and women. are rare in children. and its peak incidence o ccurs between 40 and 60 years.€Two thirds of patients are symptomatic with nonsp ecific pain, cough or dyspnea and 70% of patients exhibit a systemic disease. 10 -50% have myasthenia gravis. Recent evidence suggests that the myasthenic patien

ts exhibit a defect in the neuromuscular junction and that the thymus produces a n immune complex that inhibits synaptic transmission by masking the acetylcholin e receptors. It is uncertain the binding of thymoma with other diseases: thymic neoplasms, he matogenous aplasia, hypogammaglobulinemia, collagen vascular disease, megaesopha gus, myocarditis, and non-thymic malignancies. Histologically may be confused wi th seminoma. carcinoid and lymphoma. It is often required electron microscopy fo r this differentiation. There are no reliable histological criteria for defining malignancy. Clinically it is most important in classifying encapsulated and inv asive thymomas. Treatment: The treatment is surgical sternotomy. removing the en capsulated you and the rest of the gland. If the tumor is invasive resection is difficult due to additional treatment with radiotherapy. Chemotherapy is ineffec tive. The use of prostigmine is important postoperatively, atropine should be av oided and high doses of corticosteroids during the perioperative period is impor tant to achieve early extubation. The prognosis depends on the invasive nature o f the tumor: Invasive have a survival rate of 12.5% in the long term, while the encapsulated present survival similar to the population norm! (Mayo Clinic). 10. Clinical features, diagnosis and treatment of lymphomas. Clinical features and diagnosis 50% to 60% of patients with Hodgkin's disease have involvement of medi astinal lymph nodes at diagnosis, but clinical staging procedures showed that on ly 5% is confined to the mediastinum. Usually located in the anterior mediastinu m or hilum. Occur frequently in the third or fourth decades of life and are the second most common mediastinal mass in children. Many patients exhibit symptoms of chest pain, cough, fever or pain weight. obstruction of superior vena cava an d tracheal invasion can occur quickly. Treatment: aggressive surgical resection is not justified, because radiation therapy with or without adjuvant chemotherap y produces remission, significant and often cure. Short treatments with chemotherapy can be used to pr oduce a rapid and relieve pressure on vital structures with the vena cava, trach ea or bone marrow. The immunological manipulation of these tumors is an emerging possibility in our day. 11. What are the main germ cell tumors? - Teratoma: is the most common can be cystic or solid, containing all derived from germ cells, despite the predominance of ectodermal elements. Occur at all ages, most young a dults and about 80% are benign. Pain, cough or dyspnea are the most frequent sym ptoms. When it can be broken to bronchial expectoration of hair and sebaceous ma terial. The rupture to the pericardium can cause cardiac tamponade. The benign l esions are cured by surgery, while the teratocarcinomas have poor prognosis. - S eminoma: there are about 100 cases reported in all the anterior mediastinum. 95% occur in men between 20 and 40 years of age. Symptoms are rare and may include cough, chest pain and dysphagia. Obstruction of superior vena cava occurs in 10% of cases. The aggressive surgical resection should not be done because this is extremely radiosensitizing tumors with survival rates of up to 75% in five years , even in the presence of skeletal metastases, pulmonary and lymph node. - Chori ocarcinoma and Embryonal carcinoma: are highly malignant neoplasms in young male patients with fulminant symptoms of cough, pain and dyspnea. Gynecomastia is co mmon. The choriocarcinoma secrete human chorionic gonadotropin, and embryonal ca rcinoma producing alpha-fetoprotein antigen or carcinembriônico. On the occasion already detect metastases with fatal outcome despite the rapid radiochemotherap y. 12. What are the principal mesenchymal cell tumors? Include a diverse group a diverse group of neoplasms that originate from connective tissue, fat. smooth a nd striated muscle, blood vessels and lymphatic channels. When symptoms are obse rved, the tumor is often malignant. - Lipomas - are the most frequent mesenchyma l tumors, occupy the anterior mediastinum, are asymptomatic and surgery is curat ive. - Fibroids - These are asymptomatic, well encapsulated and can purchase large vo lumes and occupying an entire hemithorax.€More often than neurofibromas may unde rgo malignant degeneration. Benign tumors are cured by surgery. Fibrosarcomas ca

use hypoglycemia by hormone secretion and respond poorly to chemoradiation. - Me sothelioma - are most commonly pleural can be localized or spread diffusely alon g the pleura and pericardium. The mild lesions are excised easily, while the dif fuse type tumors are inoperable and resistant to palliative therapy. - Hemangiom a and Lymphangioma - capillary hemangiomas or cavernous types are the most commo n vascular tumors of the blood vessels of the mediastinum and can reach enormous proportions. Cervical lymphangiomas may extend to the anterior mediastinum. Alt hough this injury is difficult to total eradication, surgery should be attempted , as spontaneous regression has not been described and radiotherapy is ineffecti ve. - Other tumors - Myxoma mesenchymoma. leiomyoma, leiomyosarcoma, and rhabdom yosarcoma xanthogranuloma. 13. What are the main types of endocrine tumors of the mediastinum? - Intrathora cic goiter - The real is rare, mostly originamse in the neck. The real need is p robably the ectopic thyroid tissue and its blood supply is intrathoracic. Scinti graphy with I131 makes the diagnosis. The surgery is not always necessary if the lesion is asymptomatic. - Adenoma of paratireolde - They are in 10% of cases of hiperparatireoldismo. Adenomas are found in the upper mediastinum in close cont act with the thymus. Cause hypercalcemic. The recurrent cases after surgical tre atment are a diagnostic problem in general solved by arteriography or AMI inferi or thyroid. Apart from surgery is possible in high-risk patients, selective embo lization with infarction of the hyperfunctioning gland. - Carcinoid tumors - occ ur only rarely in the mediastinum. Can cause Cushing's syndrome, the production of ACTH or may be associated with a variant of multiple endocrine adenomatosis. They are locally invasive, making it difficult to complete surgical excision. Ho wever, the clinical outcome in patients with residual local or metastatic tumor is often prolonged. 14. Primary malignant tumors of the mediastinum. The primary carcinoma that orig inates in the mediastinum should be differentiated from other types of primary m alignancy of the mediastinum and contiguous involvement by bronchogenic carcinom a. In adult males occur in the anterior or middle mediastinum, resembling squamo us cell carcinoma, and are rapidly fatal. Sarcomatous tumors in children occur m ore frequently in the posterior mediastinum, and are rapidly fatal. 15. What are the main types of cysts of the mediastinum? Constitute about 20% of primary med iastinal lesions and have an equal incidence in children and adults. In adults a re usually asymptomatic; in babies can cause respiratory distress by compression of the trachea and in children can cause dysphagia by esophageal compression. T he congenital cysts should be operated even when asymptomatic, because of their tendency to infect themselves, increase volume and DD with other lesions of the mediastinum. - Pericardial cysts - occupy, in general, the angle cardiophrenic. are solitary and are usually diagnosed in adult living. Rarely communicate with the pericardium. CT and ultrasound differentiate these cysts of Morgagni hernias . - Bronchogenic cysts - are commonly found behind the carina. can be attached t o the esophagus or localized in the pericardium. Rarely communicate with the tra cheo-bronchial tree. CT and fluoroscopy may be needed to differentiate from adja cent biliary structures. The treatment is surgical. - Enteric or duplication cys ts - occur in the posterior mediastinum, is lined by epithelium of the gut and a re adjacent or pertaining to the esophagus. 6D% occur in infants. Cysts lined by gastric mucosa may show ulceration. bleeding and perforation. Rarely are multip le or are associated with abdominal duplications of other portions of the alimen tary tract. - Thymic cysts - are rare and occur as masses assintornáticas the an terior mediastinum. - Cysts of the thoracic duct - occur in the posterior medias tinum. - Nonspecific cysts - are probably benign cysts of a definite nature who suffere d inflammatory degeneration.