Diseases

of the
Mediastinum
 The mediastinum is defined as the potential space between the two
pleural cavities bounded by the sternum anteriorly, the vertebral
column posteriorly, the thoracic inlet superiorly, and the diaphragm
inferiorly .
The major mediastinal structures are the heart and great vessels,
the trachea and main bronchi, and the esophagus, all closely related
to one another and connected by loose connective tissue. Also
present are the thymus, lymph nodes, and fat.
Mediastinal diseases:

*Non neoplastic disorders:

- Pneumo mediastinum.
- Mediastinitis acute and chronic.
- Congenital cysts of mediastinum.

*neoplastic diseases: benign and malignant.

Pneumomediastinum:
Pneumomediastinum (mediastinal emphysema) is characterized by
the accumulation of air or gas in the mediastinum.
Depending on the cause, management may be expectant or may
involve specific treatment directed at the underlying abnormality.
Pneumomediastinum is frequently associated with other forms of
extra-alveolar air, including pulmonary interstitial emphysema,
pneumopericardium, pneumothorax, subcutaneous emphysema,
pneumoretroperitoneum, and pneumoperitoneum.
■ Spontaneous Pneumomediastinum
Idiopathic spontaneous Pneumomediastinum is a rare self-limited condition
that most commonly affects young adult men.
Hamman is credited with the original description of this entity in 1939,
including the characteristic crepitation synchronous with the heartbeat heard
in these patients (Hamman sign).
The majority of patients with spontaneous Pneumomediastinum have
predisposing factors that cause increase in airway pressure, which leads to
alveolar rupture.
■ Spontaneous Pneumomediastinum
Most commonly, this results from straining against a closed glottis (i.e., the
Valsalva maneuver) as during vomiting, coughing, or exercising. Other
mechanisms include sudden and/or severe increases in lung volume, as occur
during marijuana smoking, inhaling of cocaine, or during a seizure.
Localized airway obstruction from tumor, foreign bodies, asthma, or
parenchymal lung disease can also cause alveolar rupture. An accurate history
is most important to define the mechanism in a particular patient.
 Spontaneous Pneumomediastinum almost always presents with
substernal pain, often pleuritic, which may radiate to the neck or
back.
Additional symptoms that may occur, either separately or
in combination, include dyspnea, dysphagia, odynophagia, and
dysphonia.
Air in the subcutaneous tissues of the neck produces a
characteristic change in voice quality, a higher-pitched nasal tone
that the experienced clinician easily recognizes.
 Examination often reveals palpable subcutaneous emphysema in the
neck.
Auscultation of the chest may reveal a crunching or clicking sound
heard over the pericardium, synchronous with the heartbeat (Hamman
sign).
Low-grade fever is present in about one-third of cases and mild
leukocytosis in about one-half.
Nonspecific electrocardiographic changes, such as ST-T wave changes
and ST elevation, may also be present. A chest radiograph usually
demonstrates a thin radiolucent strip along a mediastinal fascial plane,
most commonly along the left heart border.
Computed tomography (CT) is more sensitive in detecting air than are
plain radiographs .Air may be evident deep in the neck as well as in the
subcutaneous tissue.
The differential diagnosis is broad and includes musculoskeletal,
pleural, pulmonary, cardiac, and esophageal causes. Although most
patients who present are not acutely ill, an occasional patient may
suffer an acute, catastrophic onset with hypotension and
hemodynamic compromise. Esophageal perforation is the condition
most likely to be confused with spontaneous mediastinal emphysema.
■ Pneumomediastinum associated with mechanical ventilation
Mechanical ventilation is commonly associated with
Pneumomediastinum and may often lead to life-threatening tension
pneumothorax.
Alveolar rupture results from high peak inspiratory pressures,
which increases alveolar pressures in patients with abnormal airways
or parenchyma (decreased compliance).
Classic predisposing factors include high tidal volumes, high levels
(PEEP), and “fighting” the ventilator.
Air trapping with (auto-PEEP) is an under recognized cause of
barotrauma.
It is not clear if one mode of ventilation (pressure-controlled vs.
volume-limited) is associated with a decreased incidence of
barotrauma.
Unlike spontaneous mediastinal emphysema, Pneumomediastinum
occurring in a patient on mechanical ventilation is potentially
catastrophic because of its frequent association with tension
pneumothorax.

The chest radiograph should be closely examined to detect even a

small pneumothorax and, if such is present, tube thoracotomy should
be promptly performed.

Obviously, a sudden deterioration marked by hypotension and

increased pulmonary pressures should prompt immediate attention
with insertion of unilateral or bilateral chest tubes, depending on the
clinical examination.
■ Pneumopericardium:
Pneumopericardium as a form of barotrauma is much more frequent
in neonates, due to immature fascial planes.
Hemodynamically significant tamponade is also much more likely to
occur in infants rather than adults and has resulted in collapse and
death. Pericardial drainage with a subxyphoid tube should be
performed promptly in the neonate. In the adult, drainage should be
performed only if there is hemodynamic embarrassment.
 Mediastinitis

acute chronic

Mediastinal Fibrosing
perforation extension
granuloma Mediastinitis

Abdominal:
Descending Out:
esophageal tracheobronchial chest Pancreatic
cervical Thoracotomy
pseudo cyst

Necrotizing
instrumental
pneumonia
Acute Mediastinitis
Acute Mediastinitis is a life-threatening disorder .
All three mediastinal compartments can be affected; the anterior
compartment most commonly after sternotomy for cardiac surgery,
the middle compartment usually from esophageal perforation, and
the posterior compartment from direct extension from the neck,
lung, or spine. Instrumental perforation of the esophagus is the
most common cause of acute Mediastinitis
Mediastinitis from esophageal perforation:

Instrumental perforation of the esophagus now accounts for

almost one-half of all esophageal perforations. Perforation is more
9

common after rigid esophagoscopy, dilation of a stricture, and

achalasia, but it also occurs after variceal sclerosis, esophageal
tube placement (nasogastric, Sengstaken), and simple flexible
esophagoscopy.
Boerhaave syndrome (postemetic rupture) was described in
patients with unexplained Mediastinitis.
Patients present with the abrupt onset of severe substernal chest
pain, which is pleuritic after forceful vomiting .
Dyspnea is common even in the absence of pneumothorax.
Shock develops quickly and the patient usually appears gravely
ill.
■ tracheobronchial perforation

Tracheobronchial perforation is rare and is most commonly seen following

trauma or instrumentation.
Less common causes include :
• anastomotic dehiscence after lung transplantation or airway surgery
and necrotizing infections involving the airway.
 Severe Mediastinitis is rare after tracheobronchial disruption,
presumably due to the less noxious nature of its contents and better
containment.
Intubation is now the most frequent cause of tracheobronchial
injury, but injury can be avoidable with gentle and proper technique.
Blood in the airway, airway obstruction (infrequent), subcutaneous
and mediastinal emphysema, and pneumothorax are the common
presenting signs.
Prompt recognition and operative repair are necessary and yield
excellent results, although small tears in the cervical trachea may
often be managed with antibiotics alone, without operation.
■ Descending necrotizing Mediastinitis:
Mediastinitis occasionally develops after severe deep head and
neck infections that originate from the oropharynx or
hypopharynx.
Most patients present with a mixed aerobic and anaerobic
infection.
Previously these infections had a fulminant, often lethal course
with mortality as high as 40%.
Extension of the cervical infection down the layers of the deep
cervical fascia into the mediastinum leads to this syndrome of
descending necrotizing mediastinitis.
Downward spread is aided by gravity, negative intrapleural
pressure, and lytic dissolution of fascia and fat.
The deep cervical fascia consists of three layers: the superficial
(buccopharyngeal) layer, the middle (visceral)
layer, and the deep layer that is further subdivided into the alar
and prevertebral layers.
All layers originate from the base of the skull. The superficial layer
terminates at the superior chest. The visceral layer terminates as a
continuation of the pericardium.
The potential space between the superficial and middle layers,
called the retropharyngeal space, allows infection to descend to the
middle of the chest.
The alar layer terminates at the diaphragm. The potential space
between the middle and the alar layer confines infection above the
diaphragm.

Rare infections between the alar and prevertebral layers may allow
the spread of infection below the diaphragm.

descending necrotizing Mediastinitis is classified according to the

extent of the infection to guide the surgical management according to
this classification: Type 1, infection above the carina, Type IIA,
infection to the level of the lower anterior mediastinum, and Type
IIB, infection involving the lower anterior and posterior
mediastinum.
The criteria for diagnosing descending necrotizing Mediastinitis
include:
(1) severe head and/or neck infection.
(2) radiographic evidence of Mediastinitis.
(3) evidence of necrotizing infection.
(4) establishment of a relationship between the cervical infection
and necrotizing Mediastinitis.
■ Mediastinitis from direct extension:

Necrotizing pneumonias may cause Mediastinitis by direct extension, most

often in immunocompromised patients. Aspergillosis of the posterior
mediastinum has been reported with increasing frequency and is highly lethal.
Treatment involves reversal of immunosuppression (if possible), appropriate
antibiotic therapy, and surgical drainage and debridement.
Pancreatitis can extend from the retroperitoneum into the mediastinum and
may present as a mediastinal process with evidence of mediastinitis.
Pancreatic pseudocysts can also erode into the mediastinum and cause pleural
effusions with increased levels of amylase. Treatment is directed at providing
adequate drainage of the pseudocyst, usually by internal drainage into the
stomach. The pleural effusion(s) may require tube thoracostomy drainage.
post sternotomy Mediastinitis:

Sternal wound infection with resulting Mediastinitis is a relatively

new entity, which emerged in the era of modern cardiac surgery.
The incidence remains low at 0.5% to 1% of all sternotomies, but
such infection is a source of major morbidity, prolonged hospital
stay, and significant mortality (0%–30%; average, 15%).
prolonged preoperative stay, advanced age, BMI >30 kg/m2, chronic
obstructive pulmonary disease, diabetes, reoperation, blood
transfusions, and reexploration for bleeding are significant risk
factors.
Staphylococcus aureus is the most common causative organism.
chronic Mediastinitis:
Several important clinical entities within the spectrum of chronic
mediastinits, including:
• mediastinal granuloma.
• fibrosing Mediastinitis.
• compression syndromes arising from fibrosing Mediastinitis.
Mediastinal granuloma:

Granulomatous mediastinitis is a disease of the mediastinal lymph nodes

usually resulting from infection by Histoplasma capsulatum and occasionally
from tuberculosis, other fungi, and sarcoidosis.
Coalescence of caseous mediastinal lymph nodes can result in a single large
mass that incites a considerable fibrotic response, which can result in
encapsulation and produce a mediastinal granuloma.
The right Para tracheal area is the most common site for development of an
encapsulated mass. When calcification
is absent and the patient presents with what appears to be mediastinal
adenopathy, a tissue diagnosis is required to exclude malignancy.
With progressive increase in the size of this “benign” mass, compression of
the trachea, superior vena cava, or esophagus can occur.
■ fibrosing Mediastinitis:

Fibrosing mediastinitis may cause a variety of clinical syndromes due to the

compression and/or obstruction of vital mediastinal structures by the dense
fibrous tissue reaction that is present.
fibrosing mediastinits is most commonly associated with Histoplasma
capsulatum infection. Other rare causes include other fungi, tuberculosis,
silicosis, sarcoidosis, the drug methysergide, autoimmune disorders, and
familial multifocal fibrosclerosis.
Fibrosing mediastinitis is labeled as idiopathic if there is no identifiable
cause.
Hypothesis that fibrosing mediastinitis results from a delayed
hypersensitivity reaction to fungal, mycobacterial, or other antigens.
Pathologic features include the presence of dense fibrotic tissue surrounding
the trachea and hila of the lungs, often extending into contiguous structures.
Compression of the airway, esophagus, pulmonary arteries, or pulmonary
veins may occur.
The involved mediastinal organs that were compressed were
vascular (48%), bronchial (27%), superior vena cava only (20%),
and esophagus (3%). Only 5% of patients were asymptomatic at
presentation.
Presenting symptoms included dyspnea (47%), cough (21%),
symptoms compatible with the superior vena cava syndrome (21%),
chest pain (20%), and hemoptysis (20%).
Focal mediastinal imaging findings were seen in 95% of patients
while only 5% had diffuse infiltration.

Calcifications were seen in 73% of patients.

PET scans, when obtained for the suspicion of malignancy, were
uniformly positive.
The optimal treatment of fibrosing mediastinitis remains unknown.
Case reports have suggested that antifungal, anti-inflammatory, or
antifibrotic treatment may have some benefit, but large case series
mostly refute these observations. Interventions to relieve compression
of mediastinal structures have been reported with moderate success,
but sometimes with relatively high periprocedure morbidity
The clinical definition of fibrosing mediastinitis was radiographic evidence of
an infiltrative mediastinal process with associated vascular, airway,
or esophageal compression. The pathologic definition was that of a
predominance of extensive pauci-cellular fibrotic tissue infiltrating
and obliterating adipose tissue with or without patchy infiltration
of mononuclear cells.
compression syndromes associated with fibrosing Mediastinitis:

1- superior vena cava syndrome.

2- trachea bronchial obstruction.
3- esophageal compression.
4- pulmonary vein obstruction.
■ compression syndromes associated with fibrosing Mediastinitis:

The most common mediastinal compression syndrome seen in

fibrosing Mediastinitis is the superior vena cava syndrome, which
occurs in 20% to 50% of patients. In the vast majority of patients,
the superior vena cava syndrome is due to malignant disease; fibrosing
Mediastinitis is the most common benign cause.
Patients present with distention of the veins in the neck; edema and plethora
of the face, neck, and arms; and central nervous system complaints such as:
headache and visual disturbances.

venous collaterals develop over the anterior chest wall and, in many
patients,
provide adequate decompression.
Surgical bypass is reserved for patients with intractable symptoms
and is performed by :
*connecting an unobstructed large brachiocephalic vein to the right
atrial appendage with a graft of either a saphenous vein or an
externally supported polytetrafluoroethylene graft. Favorable long-
term results have been reported.
*Percutaneous angioplasty and stenting of a stenotic superior vena
cava has been reported, but long-term follow-up is limited.
 A localized stenotic area sometimes can be dilated, but often pulmonary
resection is required. Resection is the procedure of choice if chronic infection
has been present.
Bronchoscopic interventions are appropriate if lung parenchyma remains
normal.
The bronchoscopic placement of stents into the trachea and/or mainstem
bronchi may allow for adequate management of a compressed airway.
A Y-bifurcation stent and individual self-expanding stents placed in the
trachea or bronchi are available.
Airway management must be individualized based on findings at
bronchoscopy.
Often the airway strictures are so fibrotic that they are not amenable to
stenting.
Complete or partial unilateral or bilateral pulmonary artery obstruction
can result from fibrosing Mediastinitis .
Congenital Cysts of the Mediastinum:
Bronchopulmonary Foregut Anomalies:
1-Bronchogenic cysts.
2-Enterogenous cysts.
3-Neuroenteric cysts.
4-Thymic cysts.
5-Pericardial cysts.
6-Thoracic duct cysts.
Anatomy:
Cysts arise in each of the three distinct anatomic regions of the
mediastinum:
• The anterosuperior compartment extends from the manubrium of
the sternum and the first rib to the diaphragm. The anterior border
of this region is the posterior sternal table, while the posterior
margin includes the pericardium and the innominate vessels.
Thymic cysts and endocrine lesions, such as thyroid goiters
and cystic adenomas of the parathyroid gland, are found in this
compartment.
The middle mediastinum is the site of origin of most
bronchopulmonary foregut cysts.
The boundaries of the middle mediastinum include the pericardial
reflections superiorly and
anteriorly and the diaphragm inferiorly.
The posterior margin of the middle mediastinum is the anterior
border of the spine.
Pericardial cysts, as well as bronchogenic cysts, are found in this
compartment.
The posterior mediastinum extends from the superior aspect of the
first thoracic vertebral body to the diaphragm.
Its anterior border is the ventral aspect of the vertebral bodies and it
extends posteriorly to the articulation of the vertebral
transverse process with each rib. The posterior mediastinum includes
both costovertebral sulci and segmental nerve roots
as well as the sympathetic chain.

Other structures found within the posterior compartment include the

esophagus, vagus nerves, the thoracic duct, the azygos vein, as well as
the descending aorta.
Neurenteric cysts, thoracic duct cysts, as well as some esophageal
duplication cysts, and bronchogenic cysts are found in this
1. BRONCHOGENIC CYSTS:
EMBRYOLOGY AND TERMINOLOGY:

The primitive respiratory and upper GIT systems have a common

embryologic endodermal origin.

The primitive foregut gives rise to pharynx and subsequently giving

rise to the larynx, upper and lower respiratory tracts, esophagus,
stomach, proximal duodenum, liver, pancreas, and associated ducts.

Throughout embryogenesis, abnormal bronchi and bronchioles may

form larger saccular structures, which are clinically recognized as
bronchogenic cysts.
Cysts abutting the trachea, carina, or hilum are termed mediastinal
bronchogenic cysts and they rarely maintain communication with
the respiratory tract.

Less frequently, cysts contained within the pulmonary parenchyma,

most commonly in the lower lobes are termed intrapulmonary
bronchogenic cysts.

Other locations include the neck, esophageal wall, pleura and

diaphragm, and, rarely, in the skin.
Bronchogenic cysts are lined by respiratory epithelium and may
contain cartilage within their walls.
PRESENTATION AND DIAGNOSIS:

15% -20% of all mediastinal masses.

77% present below the carina while
23% above the carina.
Most young patients with bronchogenic cysts have symptoms at
the time of diagnosis due to the smaller size of their thorax and more
malleable airways.
CXR : Suggest Br. Cyst. in up to two-thirds of cases The usual
appearance is that of a 2 -10 cm ovoid, smooth, homogeneous mass
that abuts the mediastinum or hilum or splays the carina.
An air–fluid level : persistent bronchial communication or secondary
infection of the cyst.
THERAPY:
Surgical excision
Thoracotomy or
Video-assisted thoracoscopic surgery (VATS).
2. ENTEROGENOUS CYSTS:

EMBRYOLOGY AND TERMINOLOGY Esophageal duplications. They

arise from the elongating esophagus, which separates from the
respiratory tract at about the 5th week of gestation.
Enterogenous cysts represent failure of normal recanalization during
embryogenesis.
Most esophageal duplications are of the closed and cystic type. Rarely
they are tubular or demonstrate preserved communication with the
alimentary tract.
PRESENTATION AND DIAGNOSIS:

Less common than br. Cysts 5% - 15%.

75% recognized before 16 years of age.
60% are located adjacent to or within the lower one-third of the
esophageal wall .

More common on the right side. 2:1.

Most adults are asymptomatic.
If left untreated most will become symptomatic.

Symptoms are usually due to compression of the airways resulting in

cough, dyspnea, and occasionally stridor. Dysphagia is infrequent.
CXR : smooth-walled, posterior mediastinal lesions at the base of the
right hemithorax.
Barium swallow : Deviation of the lumen around the cyst, but rarely
shows communication with it.
Proximal esophageal dilatation is not common because the cysts
usually are not obstructive.
CT or MRI : characterizes the contents of the cyst and its relationship
to contiguous structures.
Esophaguscopy : smooth, soft, compressible mass without mucosal
abnormality or communication with the extra luminal mass.
THERAPY
Resection:
Standard thoracotomy or VATS.
3.NEURENTERIC CYSTS:

*EMBRYOLOGY AND TERMINOLOGY : 5% of all foregut lesions.

Consistently associated with bony anomalies of the spine.( butterfly
vertebrae, hemi vertebrae and spina bifida.)
*Occur in the lower cervical and upper thoracic regions.
May be – intradural, extra medullary.
- ventral or ventrolateral to sp. cord.
PRESENTATION AND DIAGNOSIS:

All present within the first year of life.

More than 50% of affected children have neurologic complaints or
findings (back pain, motor deficits of a lower extremity, and gait
disturbance, if there is communication with the spinal canal).
The triad of a mediastinal mass, airway symptoms, and a vertebral
anomaly may be present.
CXR : medistinal mass; vertebral anomaly.
CT : myelography.
MRI : provides complete, noninvasive assessment of the bony
abnormality, the intra spinal extent of the cyst, and the degree of
compression on s.cord.
Therapy:

No intraspinal extension of the cyst : thoracoscopic excision.

Minimal involvement of the spine: resection(VATS or posterolateral
thoracotomy).
If the spinal abnormality is severe: combined neurosurgial and
thoracic approach is recommended.
4.Thymic cysts:

The thymus is derived from the third pharyngeal pouch. Its development is
incomplete at birth, continues to grow throughout childhood into adolescence.
Cysts within the gland are thought to occur during adulthood, when gland
involutes and central cells degenerate and replaced by fat..
Thymic cysts are rare.
Congenital or acquired lesions.
3% -5% of all anterior mediastinal masses.
Most thymic cysts are asymptomatic.

These cysts arise in anterior mediastinum and may extend to the middle
mediastinum.

Congenital cysts : typically unilocular .

Acquired cysts : usually multilocular.

Complete thymic excision (sternotomy, thoracotomy, VATS)

5.PERICARDIAL CYSTS:

If communication exists between pericardial sac and the extra

pericardial fluid collection : diverticulum.
Absence of communication : pericardial cyst.
Pericardial cysts are simple, smooth-walled cystic lesions. Commonly
located at the lateral basal edge of the pericardium ( at the right
cardio pherenic angle).
D D : Foramen of Morgagni hernias.
Prominent pericardial fat pads.
They can be differentiated from more solid mediastinal tumors by CT
or MRI.
No malignant potential and rarely get infected.
Complications : hemorrhage or spontaneous rupture.
Needle aspiration is often followed by reaccumulation of fluid so
not recommended.

Surgery for pericardial cysts or diverticula is not indicated in the

majority of cases because these lesions are always benign and
asymptomatic.

Resection : Symptomatic cysts.

Cysts demonstrating significant enlargement.
Right VATS : is successful in excising the cyst.
6. THORACIC DUCT CYSTS:

Congenital weakening of the thoracic duct walI leads to thoracic

duct cysts.
These cysts are exceedingly rare .
MRI is superior to CT.
To confirm diagnosis : lymphangiography.
Small cysts : observation.
Symptomatic and larger cysts : resection.
Excision : Thoracotomy or VATS.
Benign and Malignant Neoplasms of the Mediastinum:

The presenting symptoms of a mediastinal mass vary widely and are

influenced by anatomic location and the presence of malignant invasion or
mass effect.
Dyspnea or cough may result from airway invasion or abutment,
tamponade, or the presence of a pleural effusion.
Dysphagia is seen with esophageal compression, and chest pain may
represent chest wall or neural invasion or abutment.
Invasion of the airway may result in hemoptysis, invasion of the recurrent
laryngeal nerve may present as hoarseness, and invasion of the superior vena
cava can present with facial swelling and superior vena cava syndrome.
Constitutional symptoms such as fever and night sweats are often associated
with mediastinal lymphoma, and myasthenic symptoms may be suggestive of
thymoma.
Mediastinal tumors:

1-Thymic tumors.
2-Lymphomas.
3-Germ cell tumors.
4-Thyroid and parathyroid masses.
5-Mesenchymal tumors.
Thymoma:
The term thymoma : thymic epithelial neoplasm.
Seminoma, carcinoid tumor, Hodgkin and non-Hodgkin
lymphoma that can involve the thymus are not types of
thymoma.
Classificatin 0f thymic epithelial tumors:
(a) medullary thymoma.
(b) mixed thymoma.
(c) predominantly cortical (organoid) thymoma.
(d) cortical thymoma.
(e) well differentiated thymic carcinoma.
Thymomas arise in the anterior mediastinum,
Sometimes seen in the neck or extending in to the middle and
posterior mediastinum.
Unusual in the young, average age of 50-60 years.
Sex : Equal.
They are asymptomatic and present as an incidental finding in 25% of
cases
In patients with myasthenia,
10% have a thymoma.
65% have follicular hyperplasia of thymus..
25% have a normal thymus.
CT & MRI: effective for detection and assessment of extent of
thymoma.
*Thymic Carcinoid Tumor :

Malignant tumor of neuroendocrine origin,

Pathologically distinct from thymoma.
May be associated with carcinoid tumors of bronchus or
ileum. Often invades locally and tends to metastasize CT & MRI
imaging features are essentially those of thymoma.
3. Thymo lipoma : rare, benign consisting of mature adipose
tissue with islands of normal gland.
Sex : equal. Anterior mediastinum.
CT and MRI : fatty tumor with soft tissue strands representing
the areas of normal thymus.
• ■ Thymic carcinoma:

Clinical assessment and management of thymic carcinoma are:

presentation

• Thymic carcinomas (WHO type C) are highly aggressive neoplasms of

thymic epithelial origin and are very different from thymomas .

• Thymic carcinomas are rare, constituting approximately 10% of all thymic

neoplasms. They can occur at any age but are most frequently observed in
persons between 30 and 60 years of age.
• The majority of patients with thymic carcinoma present with symptoms
of local invasion or compression such as cough, chest pain, or superior
vena cava syndrome. Pericardial and/ or pleural effusions are often seen.

• Thymic carcinoma is typically not associated with myasthenia gravis.

Unlike thymomas, thymic carcinomas frequently metastasize to lymph
nodes and distant sites.
Diagnosis:

Thymic carcinomas can be distinguished from thymomas based on

their malignant histologic features and different
immunohistochemical and genetic characteristics. Imaging studies
often reveal an invasive presentation, and for this reason,
percutaneous needle biopsy should generally be undertaken.
Treatment:

While there is no standard-of-care approach to patients with thymic

carcinoma, a multidisciplinary strategy is recommended.

• stage I–III and some Iva :patients should be treated with some
surgical resection plus chemotherapy and/or radiotherapy.

• Although thymic carcinomas generally respond poorly to

chemotherapy, carboplatin and paclitaxel are recommended
because this combination has shown the highest response rates in
clinical trials.
• For the rare thymic carcinoma patients with clearly
resectable disease, surgery is considered the primary
therapeutic modality.

• unresectable or disease that invading one or more

surrounding organs or major vascular structures,
neoadjuvant chemotherapy and/or radiation may improve
operability and permit subsequent resection .

• Immune therapy used in refractory cases.

II. Germ Cell Tumors
Approximately 20% of all mediastinal mases.
A)Benign : mature cystic teratoma.
B)Malignant : seminoma and nonseminoma.
The malignant tumors have a strong predilection for male sex.
A)Mature Cystic Teratoma : Benign lesion.
Accounts for the majority of mediastinal GCT.
Sex : equal.
Often clinically silent
May be diagnosed incidentally.
Can grow to a very large size.
Sepsis or rupture into the pleural space or bronchial tree.
Plain films : show a well-defined anterior mediastinal mass with calcification
and areas of soft tissue and fat density.
CT : the wall is well defined and may contain calcification.
B) Malignant Germ Cell Tumors :
Classified into seminoma, and nonseminomatous forms
(teratocarcinoma, embryonal carcinoma, yolk sac tumor,
choriocarcinoma and mixed GCT).
Predominantly disease of young male adults.
About 70% have symptoms at presentation (dyspnea, cough, chest
pain and SVC obstruction)
Plain CXR , CT and MRI : relatively nonspecific and are similar to
those of other malignant tumors of the anterior mediastinum. Plain
films show a lobular mass, in which fat density or calcification is
absent.
CT features are those of an asymmetric, lobulated or irregular soft
tissue mass.
III. Lymphoma
Both Hodgkin lymphoma (HL) and non­Hodgkin lymphoma (NHL) can
present as anterior mediastinal masses.
HL accounts for approximately 50% to 70% of mediastinal lymphomas.
The three most common types of lymphoma found in the
mediastinum are nodular sclerosing HL, large cell lymphoma, and
lymphoblastic lymphoma.
Tends to affect young adults.
Commoner in females.
May present incidentally on imaging studies.
May cause symptoms of chest pain, cough, dyspnea or superior vena
cava obstruction.
Systemic symptoms can occur in disseminated disease.
Most patients present with asymptomatic lymphadenopathy,
but up to 25% of patients will experience B symptoms consisting of fever,
night sweats, and weight loss.
Over half of all patients with HL will have involvement of the mediastinum,
almost always in the anterior mediastinum or involving paratracheal lymph
nodes.
Suspicion for HL warrants a tissue biopsy, and the presence of Reed–Sternberg
cells is pathognomonic. Because the tumors tend to be quite fibrotic, with only
scattered malignant cells, surgical biopsy is often required to establish a
diagnosis, either by cervical mediastinoscopy (for paratracheal or middle
mediastinal nodes) or by anterior mediastinotomy (for anterior mediastinal
masses).

PET generally demonstrates high FDG avidity in lymphomas, and therefore

PET/CT may be useful in guiding sites for biopsy.
Castleman Disease:

Castleman disease is a rare lymphoproliferative disorder that can

involve enlargement of one (unicentric Castleman disease) or
multiple (multicentric Castleman disease) lymph nodes.
Most adults with unicentric disease are asymptomatic. The
mediastinum is the most common location of unicentric
disease, and lesions most frequently occur in the anterior and
middle mediastinal compartments.
Multicentric Castleman disease is :peripheral lymphadenopathy
and hepatosplenomegaly; patients also demonstrates mediastinal
and intra­abdominal adenopathy.
Multicentric Castleman disease is classified as :
- human herpesvirus 8 (HHV­8) associated, typically
occurring in immunocompromised patients
- HHV­8­negative .
- idiopathic multicentric Castleman disease.

Castleman disease is categorized into two histologic types

(hyaline vascular and plasma cell).

but the centricity rather than histology is more important in

predicting long­term outcome.
Complete surgical resection should be performed in unicentric disease and is
associated with greater than 95% overall survival and greater than 80%
disease­free survival at 5 years.

Multicentric Castleman disease can be rapidly progressive and often fatal,

and the role of surgery is primarily to obtain tissue to establish a diagnosis,
with the treatment being systemic therapy. Castleman disease can mimic
many other mediastinal tumors and must be kept in mind as a rare possible
diagnosis.
V. Mesenchymal tumors
Rare.
Arise from:
1-Adipose tissue.
2-Blood vessels.
3-Lymphatics.
4-connective tissues.
5-Mussle
Thank you