DISORDERS of The MEDIASTINUM

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THE MEDIASTINUM

 THE THORACIC SPACE THAT LIES BETWEEN


THE TWO PLEURAL CAVITIES.
 IT EXTENDS FROM THE THORACIC INLET
CEPHALAD TO THE SUPERIOR SURFACE OF
THE DIAPHRAGM CAUDAD.
 IT IS BOUNDED BY THE UNDERSURFACE OF
THE STERNUM VENTRALLY AND THE
LONGITUDINAL SPINAL LIGAMENT
DORSALLY

B.Adhiwidjaja.
Disorders of the Mediastinum

ANTERIOR MIDDLE POSTERIOR

B.Adhiwidjaja.
MEDIASTINUM

ANTERIOR

INTRATHORACIC
THYROID

POSTERIOR
MIDDLE
THYMOMAS.
Benign
Malignant.
PARATHYROID
LYMPHOMAS.
ADENOMA
Hodgkin’s disease
Lymphocytic lymphoma.
Lymphocytic/histiocytic NEUROGENIC TUMORS.
lymphoma. Neurofibroma
Histiocytic lymphoma. Neurilemoma.
TERATODERMOIDS. CYST
Neurosarcoma.
Benign. Pericardial.
Ganglioneuroma.
Malignant. Bronchogenic.
Ganglioneuroblastoma
GERM CELL TUMORS Enteric.
Neuroblastoma.
Seminoma. Chemodectoma.
Embryonal carcinoma Phaeochromocytoma.
Choriocarcinoma
Structure are in the anterior
mediastinum
 Thymus gland
 Anterior mediastinal lymph nodes
 Aortic arch

B.Adhiwidjaja.
Structure are in the middle
mediastinum
 Heart
 Pericardium
 Trachea and main bronchi
 Paratracheal and tracheobronchial lymph
nodes.
 Phrenic and recurrent laryngeal nerves.

B.Adhiwidjaja.
Structures are in the posterior
Mediastinum
 Sympathetic chain
 Vagus nerve
 Esophagus
 Thoracic duct
 Posterior mediastinal lymph nodes
 Descending aorta.

B.Adhiwidjaja.
Anterior mediastinal mass
 Thymoma
 Thyroid
 Teratoma
 Terrible ”lymphoma (‘T- cell” tumor)
 Germ cell tumor
 Hemangioma
 Fibrosarcoma
 Parathyroid adenoma
 Lymphoma
 Thymic cyst.

B.Adhiwidjaja.
VISCERAL COMPARTMENT
Tumors.
 Enterogenous cyst
 Lymphoma
 Pleuropericardial cyst
 Mediastinal granuloma
 Mesothelial cyst
 Lymphoid hamartoma
 Thoracic duct cyst
B.Adhiwidjaja.
PARAVERTEBRAL SULCI
Tumors.
 Neurilemoma- schwannoma
 Neurofibroma
 Malignant schwannoma
 Ganglioneuroma
 Ganglioneuroblastoma
 Neuroblastoma
 Paraganglioma
 Pheochromocytoma
 Fibrosarcoma
 Lymphoma

B.Adhiwidjaja.
Investigation of the
Mediastinum.
 Chest Radiographic: PA and Lateral.
 Computed Tomography.
 Magnetic Resonance Imaging.
 Radionuclide Studies.
 Mediastinal Tumor Markers.
 Mediastinoscopy.
 Video- Assisted Thoracic Surgery.

B.Adhiwidjaja.
Etiologic Factors in Acute
Mediastinitis
 Esophageal Perforation.
Iatrogenic : Baloon dilatation
Bougienage.
Esophagoscopy.
Sclerotherapy.
Spontaneous : Postemetic
Straining .
Ingestion of Foreign Bodies.
 Trauma
 Postsurgical
 Deep sternotomy wound infection
 Oropharynx and Neck infections.
 Retropharyngeal abscess. B.Adhiwidjaja.
Clinical Signs and Symptoms

 Fever
 Chest pain
 Dysphagia
 Respiratory distress.
 Cervical and upper thoracic subcutaneous
crepitus.
 Sepsis
 Hemodynamic instability and death.
B.Adhiwidjaja.
Treatment

 Mediastinitis is a TRUE SURGICAL emergency


 Correcting the primary problem.
 Debridement and drainage of the spreading
infectious process.
 Antibiotics, fluid rescuscitation.

B.Adhiwidjaja.
Mayor causes of
pneumomediastinum
 Spontaneous rupture of alveoli
 Trauma
 Rupture of the esophagus or trachea.

B.Adhiwidjaja.
Mediastinal Tumors in Adults

 Neurogenic tumors 21% Posterior


 Cyst 20% All
 Thymomas 19% Anterior
 Lymphoma 13% A/Middle
 Germ cell tumors 11% Anterior
 Mesenchymal tumors 7% All
 Endocrine tumors 6% A/Middle
B.Adhiwidjaja.
Mediastinal Tumors in Children

 Neurogenic tumors 40% Posterior


 Lymphoma 18% A/M
 Cysts 18% All
 Germ cell Tumors 11% Anterior
 Mesenchymal tumors 9% All
 Thymomas Rare Anterior

B.Adhiwidjaja.
Thymic Tumors
 Thymomas
 Thymic carcinoma.
 Tumors of neuroendocrine origin.

B.Adhiwidjaja.
Modern classification of Thymic
Tumors
 Thymomas:
Noninvasive
Epithelial predominant.
Spindle cell variant.
Lymphocytic predominant.
Mixed lymphoepithelial.
Invasive
Epithelial predominant.
Lymphocytic predominant.
Mixed lymphoepithelial. B.Adhiwidjaja.
Modern classification cont.

 Thymic carcinoma:
Low grade malignancy.
Well differentiated squamous cell carcinoma
Mucoepidermoid carcinoma.
Basaloid carcinoma.
High grade malignancy.
Lymphoepithelial like carcinoma.
Clear cell carcinoma
Sarcomatoid carcinoma
Anaplastic/Undifferentiated carcinoma.B.Adhiwidjaja.
Modern classification cont.

 Tumor of neuroendocrine cell origin:


Carcinoid tumors.
Small cell/ oat cell carcinoma

B.Adhiwidjaja.
MASAOKA Classification of
Thymoma Staging
 Stage I: Encapsulated tumor with no gross or microscopic
evidence of capsular invasion.
 Stage II: Gross capsular invasion or invasion into the
mediastinal fat or pleura or microscopic capsular invasion.
 Stage III: Gross invasion into pericardium, great vessels, or
lung.
 Stage IVa: Pleural or pericardial dissemination.
 Stage IVb: Lymphogenous or hematogenous metastasis.

B.Adhiwidjaja.
Clinical Presentation
 Locally symptomatic:
Vague chest pain
Shortness of breath.
Cough.
Superior vena cava syndrome.
Hoarseness.
Pleural or pericardial effusion
.Neuromuscular syndromes
Myasthenia gravis.
Myotonic dystrophy.
Eaton- Lambert syndrome.
Myositis. B.Adhiwidjaja.
Treatment

 Surgical Excision is the keystone of therapy.


 Radiation therapy: in stage II and III disease.
 Chemotherapy: Nonresectable stage III disease or
disseminated stage IV disease (as neoadjuvant
therapy using Cisplatin, Doxorubicin, Vincristin,
Cyclophosphamide)

B.Adhiwidjaja.
MYASTHENIA GRAVIS
 IS A NEUROMUSCULAR DISORDER OR GROUP
OF DISORDERS CHARACTERIZED: A) ABNORMAL
FATIGABILITY OF VOLUNTARY MUSCLE ON REPETITIVE
ACTIVITY.
B)DECREMENTAL RESPONSE TO REPETITIVE
STIMULATION.
C)IMPROVEMENT IN NEUROMUSCULAR
TRANSMISSION ON ADMINISTRATION OF
ANTICHOLINESTERASE DRUGS.
D)ASSOCIATION WITH ABNORMALITIES OF THE
THYMUS SUCH AS HYPERPLASIA, NEOPLASIA.

B.Adhiwidjaja.
MYASTHENIA….
E)REDUCTION IN NUMBER AND
ALTERATION IN STRUCTURE OF THE
POSTSYNAPTIC ACETHLCHOLINE RECEPTOR.
F)IMMUNOLOGICALLY : PRESENCE OF
CIRCULATING ANTIBODIES TO AChRs
and complement-mediated damage to those
receptors and by favor response to
immunosuppressive therapy, including
corticosteroids, immunosuppressive drugs and
plasmapheresis.
B.Adhiwidjaja.
Results of Treatment
Classification
 I. Complete remission
A. Without any medication
B. With medication:
1. Immunosuppresive drugs.
2. Corticosteroids.
3.Anticholinesterases.
4.Combination of 1, 2, 3.
 II. Improvement
A. Marked
B. Moderate
 III. Unchanged
 IV. Worse
 V. Dead

B.Adhiwidjaja.
Oosterhuis Classification: Global
Clinical Classification of Myasthenic
Severity.
 Class 0 : No complain, no signs after excertion or at special
testing.
 Class 1 : No disability. Minor complaints, minor signs.
 Class 2 : Slight disability, clear sings after excertion.
 Class 3 : Moderate disability, clear signs at rest
 Class 4 : Severe disability. The patient needs constant
support in daily activities. Bulbar signs are pronounced.
Respiratory function is decreased
 Class 5 : Respiratory support is needed. B.Adhiwidjaja.
Benign Mediastinal
Lymphadenopathies
 I. Mediastinal granulomatous disease.
Tuberculosis
Fungal infection
Sarcoidosis
Silicosis
Wegener’s granulomatosis.
 II . Castelman’s disease
 III. Others.
Systemic lupus erythematosus.
Infectious mononucleosis
Reactive lymph node hyperplasia.
Amyloidosis.
HIV

B.Adhiwidjaja.
Signs and Symptoms Associated with
Mediastinal Tumors.

 Superior vena cava syndrome


 Hoarseness
 Horner’s syndrome
 Dysphagia
 Stridor
 Chest pain
 Cough
B.Adhiwidjaja.
Staging System for Hodgkin’s
Disease (Ann Arbor)
 Stage I: One lymph node region on either side of the
diaphragm.
 Stage II: Two or more lymph node regions on the
same side of the diaphragm.
 Stage III: Two or more lymph node regions on both
sides of the diaphragm.
 Stage IV: Diffuse or disseminated organ
involvement
B.Adhiwidjaja.
Treatment

 Pathologic stage (PS I A): Radiation


 Stage II : Combined modality therapy (chemo and
radiation)
 Stage III and IV : Combination chemotherapy with
alternating drugs such as mechlorethamine, vincristine,
procarbazine, and prednison/ doxorubicin, bleomycin,
vinblastine and dacarbazine (MOPP/ ABVD) or ABVD
alone over a periode of 8 months.

B.Adhiwidjaja.
Mediastinal Neurogenic Tumors in
Infant and Children
 Tumors of autonomic ganglia.
Neuroblastoma
Ganglioneuroblastoma
Ganglioneuroma.
 Tumors of nerve sheath origin.
Schwannoma.
Neurofibroma.
Neurogenic sarcoma.
 Tumors of neuroectodermal origin.
Melanotic progonoma.
Askin tumor.
 Tumors of paraganglia.
B.Adhiwidjaja.
Paraganglioma.
Neurogenic Benign Tumors of the
Mediastinal in Adults
 Nerve sheath origin.
Neurilemoma.
Neurofibroma.
Melanotic schwanoma.
Granular cell tumor.
 Autonomic ganglia.
Ganglioneuroma.
 Paraganglionic system
Pheochromocytoma; biologically active.
Paraganglioma; chemodectoma; biologically active.
 Peripheral neuroectodermal tumor.

B.Adhiwidjaja.
Malignant Neurogenic Tumors of
the Mediastinal in Adults

 Nerve sheath origin.


Malignant schwannoma; neurogenic sarcoma
 Autonomic ganglia.
Ganglioneuroblastoma.
Neuroblastoma.
Primary malignant melanotic tumor of the sympathetic ganglia.
 Paraganglionic system.
Malignant pheochromocytoma.
Malignant paraganglioma.
 Peripheral neuroectodermal tumor.
Malignant small cell tumor; Askin tumor?
B.Adhiwidjaja.
Primary Seminomas of the
Mediastinum
 Friedman (1951) The most common of the
malignant germ cell tumors.
 Affect a young pts population and are potentially
curable.
 Arise from totipotential germ cells are left within
the thymus.
 Originate in the anterior mediastinal compartment.
 Microscopically identical to gonadal seminoma.
B.Adhiwidjaja.
Clinical Features
 Men in the third or fourth decades.
 Asymptomatic until compression or invasion
of the local structures.
 Cough, chest discomfort, dyspnoe, hoarseness,
dysphagia, SVC syndrome.
 hCG-beta and AFP are normal.

B.Adhiwidjaja.
Treatment
 Seminoma is a radiosensitive tumor.
 Surgical intervention at presentation should generally
be limited to a diagnostic procedure.
 Unfavorable prognostic factors for cure by radiation
include: age >35 years, SVC syndrome, cervical or
supraclavicular node involvement, hilar disease.
 Cisplatin- based chemotherapy is higly effective
against mediastinal seminoma

B.Adhiwidjaja.

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