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Mediastinal syndrome
1. الرحيم الرحمن ال بسم
MEDIASTINAL SYNDROME
Dr Tai Al Akawy
Pediatrician and neonatologist
Alexandria University Children’s Hospital
2. • Compression of mediastinal structures by any
mass gives rise to a group of symptoms known
as mediastinal syndrome
• Mediastinal masses affect patients at any age
and can be asymptomatic
3.
4. • 33% of all masses present in patients less than
15 years old
• If small, usually asymptomatic and found
incidentally
• If large, usually present with respiratory
distress
5.
6. IntroductionIntroduction
TheThe mediastinummediastinum isis the region in the chestthe region in the chest
between the pleural cavities that contain thebetween the pleural cavities that contain the heartheart
and other thoracic viscera except the lungsand other thoracic viscera except the lungs
BoundariesBoundaries
AnteriorAnterior - sternum- sternum
PosteriorPosterior - vertebral column and paravertebral- vertebral column and paravertebral
fasciafascia
SuperiorSuperior -thoracic inlet-thoracic inlet
InferiorInferior - diaphragm- diaphragm
LateralLateral - parietal pleura- parietal pleura
9. ANATOMY OF MEDIASTINUM
• It is the anatomic space that lies in the
midthorax
• It is limited by the diaphragm below and the
suprasternal thoracic inlet above.
• It contains several vital structures in a small
space,
• Abnormalities can produce important
symptoms.
10. Contents of the mediastinum
The Anterior compartment contains
• The Thymus
• Substernal extensions of the thyroid
• Parathyroid glands
• blood vessels
• Pericardium
• Lymph nodes
11.
12. Contents of the mediastinum
The middle compartment contains the
• Heart,
• Great vessels,
• Trachea,
• Main bronchi,
• Lymph nodes,
• Phrenic and
• Vagus nerves.
13. Contents of the mediastinum
The posterior compartment
• The vertebrae
• Descending aorta
• Oesophagus
• Thoracic duct
• Azygous and Hemizygous veins
• Lower portion of the vagus
• Sympathetic chains, and
• Posterior mediastinal nodes.
22. Thymoma
▪ Located in superior and / or anterior mediastinum.
▪ Can occur in all age groups but mostly seen in in adults
▪ 80 % have symptoms of myasthenia gravis
Differential diagnosis :
▪ Reactive lymphoid hyperplasia
▪ Cytological distinction between thymus and thymoma is
difficult.
▪ Follicular lymphoma
▪ PrecursorT lymphoblastic lymphoma
23. Hilum can
be seen
through
mass
this must be an anterior
mediastinal mass
because it overlaps
rather than “pushes out”
the main pulmonary
arteries
This particular example is a thymoma
24. The CT-images shows a large soft tissue mass in the anterior mediastinum, which
arises in the thymus. There is associated paratracheal adenopathy (arrow(.
25. )a) PA chest
radiograph
demonstrates a goiter
(arrow) extending
into the middle
mediastinum, causing
deviation of the
trachea to the left
(black arrowhead(.
Right-sided retrosternal goiter
26. )b) CT scan shows the mass (arrow) between the trachea and right
lung
27. Lymphoma
Lymphoma is the most
common cause of an
anterior mediastinal mass
in children and the second
most common cause of an
anterior mediastinal mass
in adults.
29. In this case, enlarged lymph nodes are seen in the right
paratracheal , hilar and subcarinal areas without thymus
involvement
30. Anterior mediastinal teratoma - A large heterogenous left anterior
mediastinal mass containing soft tissue , fatty and calcific
components. Germ Cell Tumour
33. Teratoma
▪ Most common mediastinal germ cell tumor ( 50-70 % )
– Mature teratoma : adult type tissue
– Immature teratoma: immature embryonal type of tissue;
▪ Mostly benign;
▪ Mixture of somatic tissue of 3 germ layers ; ectoderm, endoderm,
mesoderm.
▪ Immature teratoma : biphasic cell pattern ; loose aggregates of
small round cells in fibrillary matrix represents neuronal
component.
▪ Malignant transformation can occur : MC is SCC or
adenocarcinoma.
34. Embryonal carcinoma
▪ Rare type of non seminomatous germ cell tumor in young
males.
▪ Often associated with teratoma ( teratocarcinoma ),
Choriocarcinoma, or seminoma.
▪ Cytologically embryonal carcinoma is indistinguishable from
poorly differentiated carcinoma of non germ cell origin.
▪ Mitotically active tumor with malignant cells arranged in
poorly cohesive 3 D clusters , with syncitial growth pattern.
▪ Occur either as mixed or pure form. AFP levels are elevated in
all cases.
35. Yolk sac tumor / endodermal sinus
tumor
▪ Rare mediastinal tumor can occur both in paediatric ( female predilection ) as
well as in adults ( male predilection ).
▪ Commonly associated with elevated AFP levels.
▪ Coagulative tumor necrosis is abundant
▪ Schiller- Duval bodies/ Glomeruloid bodies – diagnostic are seldom seen.
▪ Identification of eosinophilic “ basal –lamina like “ substances and intra-
cytoplasmic hyaline globules ( PAS + & diastase resistant), give clue to
diagnosis.
36. Choriocarcinoma
▪ Pure medastinal choriocarcinoma is extremely rare – virtualy
non existent in children.
▪ Highly aggresive tumor.
▪ Elevated S.β HCG level.
▪ Mixture of syncytiotrophoblasts and cystotrophoblasts
against haemorrhagic background.
▪ Syncytiotrophoblastic giant cells with eosinophilic
cytoplasm , pleomorphic nuclei, prominent nucleoli,
▪ Cytotrophoblasts are medium sized cells with vaculated
basophilic cytoplasm.
39. MEDIASTINAL LYMPHADENOPATHY
• Middle mediastinum is the commonest site of intrathoracic
lymphadenopathy.
• Gross lymphadenopathy is a feature of
1)Tuberculosis
2)Histoplasmosis
3) Metastatic malignancy
4) Lymphomas
5)Sarcoidosis
40. Foregut cysts in the middle mediastinum are classified as
bronchogenic or enteric.
Bronchogenic cysts are lined by respiratory epithelium and most are
located in the subcarinal or right paratracheal area
Enteric cysts are lined by gastrointestinal mucosa and are located in
the middle or posterior mediastinum near the esophagus
41. (Right) AP radiograph shows large, smooth, homogeneous, left retrocardiac
parenchymal mass (arrows).
(Left) Axial T2 MRI shows homogeneous, well circumscribed ovoid mass (arrow) with
signal greater than CSF (curved arrow).
BRONCHOGENIC CYST
42. Enteric foregut cyst
The images show a well defined lesion in the lower mediastinum in close proximity to the
esophagus, which is typical for an enteric foregut cyst.
44. NEUROGENIC TUMOURS
There are 4 histological types.
1.NEURILEMMOMA
Benign and is classically a dumbbell-shaped mass.
compress the spinal cord and produce pressure symptoms.
2.GANGLIONEUROMA
Benign, elongated and large.
Usually occurs in children but may be found at any age.
Causes flushing,hypertension,headache,sweating,diarrhoea.
3.NEUROFIBROMA
Associated with generalized neurofibromatosis
(von Recklinghausen's disease).
4.NEUROBLASTOMA
Malignant and found frequently in children.
47. The CT-images show a calcified mass in the posterior mediastinum extending over several
vertebrae, which grows into the vertebral canal.
Neuroblastoma
48. See sharp
margin
above clavicle
Mass is in posterior mediastinum, because it remains sharply
outlined in apex of thorax, indicating that it is surrounded by lung.
This particular example is a ganglioneuroma
50. • Epithelial tumors
– Thymoma
– Thymic carcinoma
• Germ cell tumors
– Pure GCT’s : GCT’s with only one histological type
– Mixed GCT : GCT with more than one histological type
– GCT’s with somatic type malignancy
– GCT’s with associated malignancy
WHO Classification of thymic tumors and mediastinal
tumors
51. • Mediastinal lymphomas and haematopoietic neoplasms:
– B-cell lymphoma
– T-cell lymphoma
– Hodgkin’s lymphoma
– Histiocytic and dendritic cell tumors
– Myeloid sarcoma and extra-medullary acute myeloid leukaemia
• Mesenchymal tumors of mediastinum:
– Thymolipoma
– Lipoma of mediastinum
– Liposarcoma of mediastimum
– Solitary fibrous tumors
– Synovial sarcomas
52. – Vascular neoplasms
– Rhabdomyosrcoma
– Leiomyomatous tumors
• Peripheral nerve sheeth tumors
• Ectopic tumors of mediastinum
– Ectopic tumors of thymus
– Ectopic tumors of thyroid
– Ectopic tumors of parathyroid
• Metastasis to thymus and to anterior mediastinum
56. Clinical Presentation
▪ Mediastinal lesions are symptomatic in 50%-75% of patients
▪ Symptoms can be caused by local mass effects, systemic effects
of the tumor, or infection
▪ Local effects are dependent on the size and location of the lesion
and result from compression of adjacent structures
▪ Symptoms are also more common with malignant tumors
because they invade adjacent structures.
57. Organ involved Symptoms and
signs
1. Trachea, main bronchi :Stridor, dyspnoea, cough, features of
lung collapse
2. Esophagus : Dysphagia (extrinsic compression on barium
swallow)
3. Superior vena cava : Engorged non-pulsatile neck veins,
oedema and cyanosis of face, neck and arms
4. Left recurrent laryngeal nerve: Hoarseness of voice, bovine
cough
5. Phrenic nerve: Hemi-diaphragm paralysis
6. Sympathetic trunk: Horner’s syndrome
59. Superior vena cava syndrome
Clinical manifestation resulting from
partial or complete obstruction of
the superior vena cava
60. Clinical PresentationClinical Presentation
Superior vena cavaSuperior vena cava
Vulnerable to extrinsic compression andVulnerable to extrinsic compression and
obstruction because it is thin walled andobstruction because it is thin walled and
its intravascular pressure is lowits intravascular pressure is low
Bronchogenic carcinoma and lymphomaBronchogenic carcinoma and lymphoma
are the most common etiologiesare the most common etiologies
61. Superior vena cava syndromeSuperior vena cava syndrome
Results fromResults from the increase venous pressurethe increase venous pressure
in the upper thorax , head and neckin the upper thorax , head and neck
Characterized by dilation of the collateralCharacterized by dilation of the collateral
veins in the upper portion of the bodyveins in the upper portion of the body
Edema and plethora of the face, neck andEdema and plethora of the face, neck and
upper chest, edema of the conjunctivaupper chest, edema of the conjunctiva
Cerebral symptoms such as headache,Cerebral symptoms such as headache,
disturbance of consciousness and visualdisturbance of consciousness and visual
distortiondistortion
62.
63.
64. Diagnostic Evaluation
• RADIOLOGY
– Plain chest x-ray taken in two planes,
posteroanterior and left lateral basic
information about the location of the mass within
the mediastinum
– Diaphragm fluoroscopy to evaluate paradoxical
motion of the diaphragm on rapid inspiration
indicative of phrenic nerve paralysis
65. • USG :
– via percutaneous route ; it provides both
spatial orientation as well as real time
monitoring with out exposure to radiation
– Via bronchoscopy / endoscopy via
oesophagus.
Approach to mediastinum
66. RADIOLOGY
–CT of the chest has replaced plain chest
radiography as the diagnostic procedure of
choice for mediastinal masses
–CT : locating small lesion in thoracic inlet,
hilum and middle mediastinum , via
supraclavicular, suprasternal and
parasternal approach. But continues real
time monitoring is not possible without
radiation exposure.
67. –MRI may enhance the diagnostic abilities of
chest CT
–Echocardiography and positron emission
tomography(PET) have been used
invasive thymomas and lymphoma
69. Lymphoma is the most common cause of an anterior
mediastinal mass in children
70.
71.
72.
73.
74. Histologic
–FNA or needle biopsy with CT guidance of a
mediastinal mass may provide sufficient
tissue for diagnosis of thymic carcinoma or
other defined neoplasms
–Mediastinoscopy, or intrathoracic biopsy
may be considered for lymphomas in
particular, and thymomas and neural
tumors
75. The biopsy can be obtained through
• Traditional bronchoscopy or
• Echo-guided endoscopy,
• Superficial node biopsy,
• Mediastinoscopy,
• Mediastinotomy,
• Transthoracic needle biopsy,
• Thoracoscopy,
• Cervical or supraclavicular biopsies;
• Thoracotomy and sternotomy are rarely
indicated
76.
77. Diagnosis requires combination of clinical, radiological, biochemical, and
cytomorphological information.
• Age :
In children : commonest thymic tumor is NHL. Thymoma is extremely
rare in childhood.
Adults : mets and benign mediastinal cyst of celomic origin or GI origin.
In adults : thymoma represents commonest primary thymic tumor,
followed by mediastinal lymphoma.
Approach to mediadiastinal tumors
Step 1 : clinical historyStep 1 : clinical history
78. • located in which part of mediastinum
– Extremely useful in diagnosis of tumors
– Depending on predominant cell morphology
diagnosis can be approached.
Step 2 : radiological localisationStep 2 : radiological localisation
Step 3 : cytomorphologyStep 3 : cytomorphology
79. History and relevant information for diagnosisHistory and relevant information for diagnosis
82. Supportive Care
Elevate the patient’s head to decrease the
hydrostatic pressure and thereby the congesion
Oxygen
Glucocorticoid therapy (dexamethasone, every 6
hours)
Glucocorticoids reduce the tumor burden in
lymphoma and thymoma and are therefore more
likely to reduce the obstruction
Loop diuretics are also commonly used
83. Chemotherapy is used in lymphomas,
small-cell lung cancer and germ cell
tumors.
Besides chemotherapy,
Radiotherapy is used to shrink the tumor
mass
84. Some caSeS muSt be approached
aS an emergency
Acute lifethreatening presentation is the
only situation in which radiotherapy before
histological diagnosis can be considered.
However, this approach should be avoided, whenever
possible.
RT prior to biopsy may obscure the histologic diagnosis.
85. Current guidelines stress the importance
of accurate histologic diagnosis prior
to starting therapy,
and the use of endovascular stents in
severely symptomatic patients to
provide more rapid relief than can be
achieved using RT.
Kvale PA, Selecky PA, Prakash UB, American College of Chest Physicians.
Palliative care in lung cancer: ACCP evidence-based clinical practice
guidelines (2nd edition). Chest 2007; 132:368S.
86. Important exceptions to this general approach
are pts who present with stridor due to central
airway obstruction or severe laryngeal edema,
and those with coma from cerebral edema.
87. These situations represent a true medical
emergency, and these patients require
immediate treatment (stent placement and
radiotherapy) to decrease the risk of sudden
respiratory failure and death.
88. Radiation therapy
RT provides considerable relief by reducing
tumor burden
Symptomatic improvement is usually apparent
within 72 hours.
91. PA and lateral chest films show
a large anterior mediastinal
mass causing narrowing and
rightward deviation of the
trachea.
92. CT exam show a low
density mass in the
anterior mediastinum with
irregular walls with calcium
in it.
Dx Teratoma, Anterior
Mediastinal
93. Three year old male with
an incidentally noted chest
mass
94.
95. single slice from an enhanced chest CT exam shows the mass to be
non-enhancing, posterior to the right bronchi, and next to the esophagus.
Dx: Esophageal Duplication
96. Eleven year old male with
upper respiratory
symptoms and wheezing
97.
98. Slice from an enhanced chest CT exam shows a multi-loculated non
enhancing mass in the anterior mediastinum
Dx-Thymic Cyst
99. Twelve year old female with a
chest symptoms and some
neck swellings
Fever ,night sweating ,loss of weight
100. PA chest films show a large, lobulated anterior mediastinal mass
displacing the trachea to the right.
101. A chest CT exam shows the mass to extend from the neck to the
diaphragm, compressing the tracheal and left mainstem bronchus leading to
left lower lobe atelectasis. The chest wall mass is partially eroding the
sternum.
Dx: Lymphoma, Hodgkin, Anterior Mediastinal, Sternal
Involvement
102. Fourteen year old male
presented with chest pain,
cough, dyspnea, hoarseness,
and superior vena caval
syndrome
103. Two contiguous slices from
an enhanced chest CT show
a homogenous, solid,
anterior mediastinal mass
and a large right pleural
effusion
Dx-Lymphoma
Non-Hodgkin,
Anterior Mediastinal
107. There are multiple causes of
mediastinal masses, but the
differential diagnosis can be
narrowed based upon the
compartment involved ,the clinical
presentation , appearance of the
mass on CT scan and tissue diagnosis
108. REFERENCES
• 1. Wright CD, Mathisen DJ. Mediastinal tumors: diagnosis and treatment. World J Surg
2001;25:204–9.
• 2.Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastinal tumors:
a 40 year experience.J Thorac Cardiovasc Surg 1971;62:379–92.
• 3. Fraser RS, Pare JA, Fraser RG, et al. The normal chest. In:Fraser RS, Pare JA, Fraser RG, et al,
editors. Synopsis of diseases of the chest. 2nd ed. Philadelphia: W.B. Saunders;1994:1-116.
• 4. Fraser RS, Muller NL, Colman N, et al. The mediastinum.In: Fraser RS, Muller NL, Colman N,
et al, editors. Fraser and Pare’s diagnosis of diseases of the chest. 4th ed. Philadelphia:W.B.
Saunders; 1999:196–234.
• 5. Park DR, Pierson DJ. Tumors and cysts of the mediastinum.In: Murray JF, Nadel JA, editors.
Textbook of respiratory medicine. 3rd ed. Philadelphia: W.B. Saunders;2000:2123-37.
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DM, editors. Imaging of diseases of the chest. 3rd ed. London: Mosby; 2000:789-892.
• 7. Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastinal tumors:
a 40 year experience.J Thorac Cardiovasc Surg 1971;62:379–92.
• 8. Davis RD Jr, Oldham HN Jr, Sabiston DC Jr. Primary cysts and neoplasms of the
mediastinum: recent changes in clinical presentation, methods of diagnosis, management,
and results. Ann Thorac Surg 1997;44:229–37.
109. • 9. Strickler JG, Kurtin PJ. Mediastinal lymphoma. Semin Diagn Pathol 1991;8:2–13.
• 10. Keller AR, Castleman B. Hodgkin’s disease of the thymus gland. Cancer 1974;33:1615–23.
• 11. Costello P, Jochelson M. Lymphoma of the mediastinum and lung. In: Taveras JM,
Ferrucci JT, editors. Radiology:diagnosis, imaging, intervention. Philadelphia: J.B. Lippincott
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of 80 cases. Br J Surg 1999;911:77-2
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correlation. Radiographics 1992;151:12-68.
Notas del editor
Mature teratoma shows : squamous epithelium, hair shaft, respiratory epithelium, chondromyxoid stroma which is better demonstrated with diff stick ( MGG ) stain- Metachromatic colour.
S-D bodies are formedd due to invagination of tumor cells and capillaries in to cystic space.
Haematogenous spread , pt present with features of gynacomastia.
Practically speaking – the ability of FNAC in separating various non semonomatous germ cell tumors, specially between embryonal carcinoma, yolk sac tumor, and choriocarcinoma are rather limited. There is often morphological overlap and its not uncommon to find mixture of various germ cell tumor components with in single lesion.
MG : myasthenia grevis , HSM : haepato-spleenomegaly. Taking the anatomic location of the lesion and age of the patient in to consideration, a narrow list of DD can be produced even before FNAC. clinical H/o of MG associated with thymoma .
past H/o of malignancy .
lymphadenopathy, HSM, others .