Dika -Mediastenal Masses

8/10/2019 Dika -Mediastenal Masses

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NORMAL ANATOMY


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Mediastinal ContentsCompartment Main Strictures

Anterior Fat, lymph nodes, thymus, heart, ascending aorta

Middle Trachea, bronchi, lymph nodes, oesophagus, descendingaorta

Posterior Para vertebral soft tissues

Mediastinal Masses

Compartment % MalignantAnterosuperior 59

Middle 29

Posterior 16


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Anatomy of the MediastinumNormal Contents Anterosuperior : thymus, extrapericardial

aorta and branches, IVC, SVC, lymphatic tissue Middle : heart, intrapericardial great vessels,

pulmonary hila, pericardium, trachea

Posterior : esophagus, vagus nerves, thoracicduct, sympathetic chain, descending thoracicaorta, azygous venous system


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History and PhysicalExamination Chest pain or dyspnea may be reportedsecondary to associated pleural effusions,cardiac tamponade, or phrenic nerveinvolvement.

Occasionally, hoarseness because of leftrecurrent laryngeal nerve involvement .

Systemic Sx present in 50% of patients anda lymphoproliferative disorder, compared with only 29% of patients with other masses(such as thymic or neurogenic lesions).

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Pain Cough

Hemoptysis SVC syndrome Hoarseness Dyspnea Horner s syndrome

Dysphagia Pleural effusion Stridor

Myathenia Gravis Phrenic nerve palsy Chylothorax

Symptoms and Signs


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Diagnosis Chest PA & Lateral Bucky film

Chest CT Fluoroscopy Bronchoscopy Esophagogram

NAB Isotope Scanning

Angiography Thoracotomy VATS Medistinoscopy


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Clinical Presentation Asymptomatic mass

Incidental discovery most common 50% of all mediastinal mass are asymptomatic 80% of such mass are benign More than half are malignant if with symptoms


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Clinical Presentation Effects on Compression or invasion of adjacent tissues Chest pain , from traction on mediastinal mass, tissue

invasion, or bone erosion is common Cough , because of extrinsic compression of the trachea or

bronchi, or erosion into the airway itself Hemoptysis, hoarseness or stridor Pleural effusion, invasion or irritation of pleural space Dysphagia, invasion or direct invasioin of the esophagus Pericarditis or pericardial tamponade Right ventricular outflow obstruction and cor pulmonale


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Clinical Presentation Superior vena cava Vulnerable to extrinsic compression and obstruction because it is

thin walled and its intravascular pressure is low, and relativelyconfined by lymph nodes and other rigid structures

Superior vena cava syndrome Results from the increase venous pressure in the upper thorax , headand neck characterized by dilation of the collateral veins in the upper portion

of the head and thorax and edema oand phlethora of the face, neckand upper torso, suffusion and edema of the conjunctiva and cerebralsymptoms such as headache, disturbance of consciousness and visualdistortion

Bronchogenic carcinoma and lymphoma are the mostcommon etiologies


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Clinical Presentation Hoarseness, invading or compressing the nerves Horners syndrome, involvement of the sympathetic

ganglia Dyspnea, from phrenic nerve involvement causing

diaphragmatic paralysis Tachycardia, secondary to vagus nerve involvement

Clinical manifestations of spinal cord compression


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Clinical Presentation Systemic symptoms and syndromes Fever, anorexia, weight loss and other non

specific symptoms of malignancy andgranulomatous inflammation


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10/21/2014Anterior mediastinal masses Benign

ThymomaThymic cystThymolipoma

Thymic hyperplasiaThyroidTeratomaCystic hygromaParathyroid adenoma

Foramen of morgagnihernia

MalignantThymic carcinomaThyroid carcinomaSeminoma

Mixed germ cellLymphomaThymic carcinoid

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Anterior Mediastinal Masses: (4 T's)(30% of mediastinal masses)

Thymoma Teratoma Thyroid (Ectopic) (Terrible) Lymphoma


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Middle Mediastinal Masses (A + B) (30%of mediastinal masses) Adenopathy (infection [bacterial,

granulomatous], neoplasm [leukemia /lymphoma, metastases])

Bronchopulmonary foregut malformations(Esophageal duplication cyst, bronchogenic cyst,

sequestration)


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Posterior Mediastinal Masses: (N) (40%of mediastinal masses) Sympathetic ganglion tumors: neuroblastoma,

ganglioneuroblastoma, ganglioneuroma (95% ofposterior mediastinal masses)

Neurofibroma Neurenteric cyst

Extramedullary hematopoesis Paravertebral soft tissue mass from infection


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Approach/Discussion: PA and lateral chest films are the first step in

distinguishing from which mediastinal

compartment the mass is arising from. Computed tomography or magnetic resonanceimaging is the next step, better characterizingthe nature and extent of the lesion, thusnarrowing the differential diagnosis. MRI isespecially good at looking for spinal canalinvasion in posterior mediastinal masses

Tissue biopsy is required for definitive diagnosis,and surgical resection for definitive cure.


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Thymoma (Staging) Stage I : contained within an intact

capsule Stage II: extension through thecapsule to surrounding fat, pleura,pericardium

Stage III : Intrathoracic metastasis Stage IV: Extrathoracic Metastasis


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Thymoma(Treatment) Stage I : Surgical resection Recurrence 2-12% Stage II & III : Surgery + Radiotherapy Stage IV : Multimodality Induction

chemotherapy, surgery + post op Radiotherapy 5-year Survival 12 54 %, not affected by the

presence of Myasthenia Gravis


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Lymphoma Metastatic is most common

5-10% is mediastinal primary Second moost common AnteriorMediastinal Mass in Adults

Malignant > Hodgkins Dx: Mediastinoscopy, thoracotomy NAB : Usually not confirmatory


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Germ Cell Tumors

Anterior Mediastinal location Mainly in late teens 15 %of Ant. Med. Tumors

in Adults, 24 % in children 1/5 is Malignant Cystic Teratoma(Dermoid Cyst) vs. Solid

tumor (Teratoma) Solid tumor : 1/3 malignant Radiosensitive

Teratoma, Malignant teratoma,Seminoma(dysgerminomas)


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Substernal Thyroid Tissues

Develops from cervical goiter or

intrathoracic remnants Can be diagnosed without biopsy by

Radioactive iodine scan

No treatment unless symptomatic,usually pressure symptoms


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Neurogenic Tumors Posterior mediastinal location 1/5 of mediastinal tumor Originate in neural crest Ganglioheuroma : most common in the

textbook Neurilemmoma most common in Korea :

Dumb bell Tumor , neural sheath origin


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Poosterior Mediastinal Tumor

( Neurillemmoma) )

Dumb - bell Tumor


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Mesenchymal Tumors Lipoma, Fibroma, Mesothelioma Superior or Anterior mediastinal location Diagnosis with CT scan May cause Hypoglycemia


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Mediastinitis Acute : endoscopy complication,

Boerhaave s SD, operation, esophagealrupture, median sternotomy

Chronic : Tbc, histoplasmosis, silicosis,fibrosing mediastinitis


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Pneumomediastinum Spontaneous : mainly in young male

adults Hamman sign Present along the Left sternal border Substernal pain, cough, Dyspnea,

Dysphagia


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Pericardial Cyst Thin-walled, mesothelial cell lining

most common in Right C-P angle Simple cysts are almost always

asymptomatic

Rare cardiac impingement


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Bronchogenic Cysts 30 - 60% of all mediastinal cysts Lined by ciliated respiratory epithelium May contain cartilages or mucous Communicate with tracheobronchial trees May become infected Wheezing, dyspnea, recurrent pulmonary

infections

A A


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A AINVESTIGATIONS

Hemoglobin, hematocrit, and white bloodcell count Red blood cell aplasia is found inapproximately 5% of patients with thymomaand manifests as a normochromic-normocyticanemia. Although rare, neutropenia can befound in association with thymomas.

Gamma globulin levelsHypogammaglobulinemia is associated with

some cases of thymoma


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LABORATORY INVESTIGATIONS

Alpha-fetoprotein AFP almost always is elevated in individuals

with non seminomatous germ cell tumors. Elevation of the AFP level is not found in

individuals with pure seminoma.

Lactate Dehydrogenase


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Adrenocorticotropic hormone (ACTH)levels The thorax should always beinvestigated for the source of ectopicACTH production. A neuroendocrine orcarcinoid tumor of the thorax should beexcluded. These tumors occur in the

mediastinum, particularly in the thymusgland, and in the lung.

Antidiuretic hormone levels : These maybe elevated with some neuroendocrine

LABORATORY INVESTIGATIONS


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ThymomaMost common tumor of the anteriormediastinum.Usually in adults with a median age of 50 years

with no gender preference.40% of patients have PNS such as MG (30%),pure red cell aplasia, or hypogammaglobulinemia(5%-10%).Other PNS, such as SLE, Cushing's syndrome,and SIADH.

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ThymomaMG is most frequent in , and symptoms includediplopia, ptosis, dysphagia, weakness, and fatigue. Diagnosis:

Serum anti-acetylcholine receptor antibody testeven if they are asymptomatic. CT-Chest with contrast.

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Noninvasive Thymoma : well encapsulated,rounded or slightly lobular shape, and usuallymanifest as a solid lesion with areas of hemorrhage,necrosis, or cystic degeneration.

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Invasive thymomas: infiltration of the adjacentstructures, irregular margins, areas of lowattenuation, and multifocal calcification.

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Germ cell tumorsClassification:Benign tumors (80%) are mature teratomas,Malignant include:

Seminomas.Nonseminomatous dysembryomas.Malignant (immature) teratomas

Associated with Klinefelter's syndrome in 8% of

cases and hematologic malignancies.Testing of serum -fetoprotein and ( -HCG) ismandatory

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TeratomaThey are well delimited in relation to thesurrounding mediastinal structures and may becystic.

Incidentally discovered. However, they can reach aremarkable size and can give rise to local symptoms.Histologically, mature teratomas consist ofirregularly arranged , well-differentiated adult tissuesof ectodermal, mesodermal and endodermal origin.

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TeratomaCT: Well-defined, smooth or lobulated margins.They are encapsulated and display heterogeneousattenuation due to the combination of soft tissue,fluid, fat, and calcific components.They are typically multilobulated cystic tumors with walls of varying thickness. Cough productive of hairor sebum is a pathognomonic sign of rupture into thetracheobronchial tree.

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Teratoma Immature teratomas are made up of the samedifferentiated tissues as mature forms inassociation with poorly organized fetal-typetissue.In childhood, prognosis is good, whereas at anyother age, their behavior is often aggressive.

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Teratoma Teratomas with malignanttransformation , teratocarcinomas, containa malignant component, most commonlysarcoma.These tumors tend to be larger than

benign forms and are often found toinvade adjacent structures at the time ofdiagnosis.

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LymphomaIs a relatively common mediastinal tumor.20% of all mediastinal tumors in adults and50% in children.

They are mostly Hodgkin's lymphomas andseldom are confined only to themediastinum at diagnosis.The most common variants of non-Hodgkin's lymphomas that primarily affectthe anterior mediastinum are large B-celllymphoma and lymphoblastic lymphoma

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Bronchogenic CystsResult from abnormal budding of the trachealdiverticulum between the 3 rd &6th wks ofgestation.

5% to 10% of all mediastinal lesions.Usually found adjacent to the tracheobronchialtree but can be also found in the posteriormediastinum or within the lungs.

Cause symptoms in adults in 30% to 45% ofcases

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Bronchogenic Cysts At CT, well-defined, round masses with ahomogeneous density similar to water.However, density and the heterogeneous aspectcan make diagnosis difficultIf there is a direct communication with thetracheobronchial tree, air-fluid levels can beseen.

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Pericardial CystsBenign intrathoracic lesions and constitute 7% ofall mediastinal tumors.Typically located at the right cardiophrenic angle

(50%-70%) or at the left cardiophrenic angle(30%-40%), or rarely in other mediastinallocations not adjacent to the diaphragm.Their size varies from a few centimeters to 30

cm.They are usually congenital but may also beacquired after cardiothoracic surgery.

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Pericardial Cysts Most pericardial cysts are asymptomatic . Patients may present with:

Chest discomfortDyspneaCoughLife-threatening complications such as cardiac

tamponade have been reported .

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Pericardial Cysts

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NeuroblastomaHighly malignant.Most common extracranial solid malignancy inpediatric patients.The most common intrathoracic malignancy ofchildhood.

Adrenal gland is a common primary site, but14% of all neuroblastomas arise in the thorax, where the tumors are commonly associated withextension into the spinal canal and osseous

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Neuroblastoma Is not as recalcitrant to chemotherapy andsurgical resection as are other chestmalignancies.They are more likely to be resectable, with lessinvasion of surrounding organs.>1/2 occur in children

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Dika -Mediastenal Masses