Mediastinal tumor

Zuzannawati, Erwin Arief *

Sub-Section of Pulmonology, Department of Internal Medicine
Universittas Medical Faculty of Hasanuddin
INTRODUCTION
Mediastinal tumor is a tumor that is contained within the mediastinal cavity is
located between the right and left lung. The mediastinum is an important part of the
thorax. The mediastinum is the central area of the chest cavity between the pleural
cavity located between pleuralis cavity and contains many vital organs and vital
structures. [ 1 ]
Important structures contained in it are the heart, arteries, veins, trachea,
thymus gland, nerves, connective tissue, lymph nodes and ducts. Broadly speaking,
the mediastinum is divided into 4 important parts of the superior mediastinum, the
anterior, posterior and medial mediastinum. Important processes involving the
mediastinum include emphysema, infection, bleeding, and many types of primary
tumors and cysts. Mediastinal cavity is narrow and can not be expanded, the
resolution of the tumor can press on nearby organs and can cause life-threatening
severity. Most mediastinal tumors grow slowly so patients often come after a tumor is
large enough, with complaints and symptoms due to tumor suppression surrounding
organs. [ 2 , 3 ]
Epidemiology
Several reviews of the mediastinal mass shows the variation in incidence
and clinical presentation depends on the age of the patient and the specific area of the
mediastinum. In adult individuals, mediastinal tumors are the most common
thymoma followed by neurogenic tumors, lymphomas, and germ cell tumors. [ 4 ]
Based on a retrospective study from 1973 to 1995 in New Mexico, USA
acquired 219 patients were identified malignant mediastinal tumors of 110,284
patients of primary malignant disease, most types of lymphoma was 55%, 16% germ
cells, thymoma 14%, 5% sarcoma, neurogenic 3% and 7% other types. [ 5 ]
While the frequency of data mediastinal tumor in Indonesia, among others,
obtained from SMF Thoracic Surgical Friendship Hospital, Jakarta. In the years 1970
- 1990 at the Friendship Hospital performed surgery on 137 cases. The study was
conducted with a cross-sectional survey method through the perpetually retrospective
medical record in the hospital over the next 5 years (January 2002-December 2006).
Obtained a total of 184 cases with neoplastic group were recorded by examination of
the PA during the years 2002-2006 as many as 164 cases. The number of samples
collected through medical records of 98 people. Based on the obtained sex men
numbered 69 (70.4%) and 29 women (29.6%). Respiratory symptoms in 87
mediastinal tumors with complaints of cough 68 (78.1%), shortness of breath 60
people (68.9%), chest pain, 39 (44.8%), wheezing sound 2 (2.3% ). 59 nonrespiratory symptoms with weight loss 45 (76.2%), fever 27 (45.7%), fatigue 16
(27.1%), other 37 (62.7%). 8 of 98 people came without complaint. Abnormalities
associated ith suppression or tumor invasion into surrounding organs 2 (2.1%),
superior vena cava syndrome, 25 (27.1%), pleural effusion 25 (27.1%) and
myasthenia gravis 28 people (36 , 3%). Tumor diagnosis based PA; thymoma 37

(37.8%), germ cell tumors 10 (10.2%), teratom 20 people (20.4%), seminoma 4

(4.1%), nonseminoma 1 (1%), lymphoma 13 (13.3%), mediastinal malignancies other
10 (10.2%) and benign tumors of the mediastinum 3 (3.1%). Based on chest
radiographs, 92 anterior mediastinal tumor, 4 medium, and 2 posterior. Data Hospital
Dr. Soetomo describes the location of the tumor in the anterior mediastinum 67% of
cases, 29% of the mediastinum medial and posterior mediastinum 25.5%. [ 4 , 6 ]

CLASSIFICATION
Mediastinum based on the division of anatomy divided into four:
1. Superior mediastinum, from the door on the chest cavity to the thoracic vertebrae
and the bottom of the sternum: Struma, parathyroid adenoma and lymphoma
2. Anterior mediastinum, from the boundary line to the superior mediastinum
diafargma in front of the heart: Struma, teratoma, thymoma, parathyroid
adenoma, lymphoma, fibroma, hemangioma Limfangioma, and Morgagni
Hernia
3. Posterior mediastinum, the superior mediastinum from the boundary line to the
diaphragm in the back of the heart: neurogenic tumors, fibrosarcoma,
lymphoma, aneurysm, kondroma, bochdalek hernia.
4. Mediastinum medial (middle), of the boundary line in the superior mediastinum to
the diaphragm between the anterior and posterior mediastinum: bronchogenic
cyst, lymphoma, pericardium cysts, aneurysms, and hernia. [ 2 ]
The division of the mediastinum into the different cavities assist in
diagnosis. This type of tumor in the mediastinum cavity can be either benign or
malignant tumors with different management and prognosis, therefore diagnostic
procedures play a pivotal role. Most cases come to the gravity or severity of
cardiovascular breath, a condition that causes the diagnostic procedures had to be
postponed for troubleshooting kegawatannya first. [ 2 , 4 ]
Mediastinal tumor classification based on the organ / tissue origin of the
tumor or histological type, as suggested by Rosenberg
Table. 1 Classification of mediastinal tumor by histopathologic

A. thymoma
Thymoma is a primary tumor in the anterior mediastinum are most
commonly found. Men and women are equal number of events and most often at the
age of 40 years. Thymoma is rare in children and adolescents. Thymoma is a tumor
or a benign epithelial tumor with a low degree of malignancy and is found in the
anterior mediastinum. Thymoma including slow-growing type of tumor. [ 5 , 7 ]
Frequent local invasion into surrounding tissues but rarely metastasizes
outside the thorax. Most are asymptomatic. If patients present with the common
complaint was chest pain, coughing, tightness or other symptoms associated with
invasion or tumor suppression to the surrounding tissue. One or more signs of the
syndrome paratimik often found in patients with thymoma, such as myasthenia
gravis, hipogamaglobulinemi and red blood cell aplasia. The incidence of
approximately 30-50% Myastenia gravis in patients with thymoma. 25% of patients
with thymoma with myasthenia myastenia are young women. Can be diagnosed with
an increase in antibody titer antiacetylcholine receptor. 10% of patients with
hipogammaglobulinemia tymoma, 5% of thymoma patients with red cell aplasia. [ 4-6
]

Radiology picture of the thorax x-ray posteroanterior lobular mass

appeared in the anterior-superior left parahillar opaque area. In the lateral position
showed a mass in the anterior portion of the thoracic cavity and the retrosternal area
that is not clean. On CT scan usually manifests as soft tissue in the anterior
mediastinum, size can be of various kinds, with smooth and firm boundaries. [ 8 ]
From the description of anatomic pathology is difficult to distinguish
benign or malignant thymoma. Definition of malignant thymoma (invasive) if the
tumor is microscopic (histopathological) and macroscopic invasive been outside the
capsule or surrounding tissue. [ 9 ]
Table 2. Classification of histological thymoma (7)

Table 3. Masaoka staging system based

Noninvasive thymoma is still limited to the thymus gland and has not
spread to other organs. All tumor cells contained or encased by a capsule and
microscopically invisible invasion of the capsule. If the tumor cell invasion has
reached the capsule then categorized invasive thymoma (malignant thymoma).
Data at the Friendship Hospital of 31 surgical cases in 1992 to 1999 cases
of invasive categorized amounted 90.3% and only 9.7% of cases are diagnosed
noninvasive or stage I. 2000-2001 data from 12 patients with thymoma were
dissected no cases of noninvasive. [ 1 ]
Data in Section of Thorax and Cardiovascular surgery in patients with
thymoma Torino University operated from years 1958-1988 (30 years), 107 patients
with 134 men and women aged between 16-76 years found 52 of 241 patients
(21.6%) died. 7 postoperative, 5 people died with myastenia gravis. 11 people
without myastenia gravis. 80% survive for 5 years, 75.6% over 10 years. Patients
with stage I thymoma good prognosis, whereas in stage II, III and IVa poor
prognosis. [ 10 ]

B. Germinal Cell Tumors

Germ cell tumor composed of seminoma tumors, teratomas and
nonseminoma. Germ cell tumors in the mediastinum are less common than thymoma,
more frequently in males and young adults an average age of 27 years.
Approximately 10-15% of cases of anterior mediastinal tumor. Most cases are a
primary tumor in the testes so that when the diagnosis was mediastinal germ cell
tumors, it must be ensured that the primary in the testis has been removed. Most
locations in the anterior (superoanterior) mediastinum. Histologically the tumor in the
mediastinum together with germ cell tumors in the testes and ovaries. Serologic
evaluation of -fetoprotein (AFP) and -human chorionic gonadotropin (-HCG) to
help evaluate patient-psien suspected malignant germ cell tumors. [ 5 , 6 ]
Teratomas are germ cell tumors are the most common followed by
seminoma tumors can form cysts or solid or a mixture of both composed of germ cell
layers, namely ectoderm. mesoderm or endoderm. Mature teratoma is the most
common mediastinal germ cell tumors and are usually benign. The tumor is not as
testicular teratoma metastatic potential and can be surgical resection. Therefore, the
anatomical location and pascaoperaif intraoperative complications can affect
morbidity due to intrathoracic structures usually involved. [ 11 ]
Intrathoracic teratoma usually appears in the cavity is very rare in the
mediastinum and lung. Most of these tumors are benign, although there are
malignant. The tumor usually found in the mid-line of the body. Symptoms can
appear in case of mechanical effects such as chest pain (52%), hemoptysis (42%),
cough (39%), shortness of breath, or symptoms associated with recurrent
pneumonitis. Other respiratory symptoms are trikoptisis (trichoptysis) (13%) are in
the productive cough sputum containing hair or sebaceous gland secretions. This
would occur if there is a relationship between the mass of the tracheobronchial
tumors. Other symptoms of superior vena cava syndrome or lipoid pneumonia.
Mediastinurn teratomas are usually discovered by accident in the photo the piston. [ 5
,
12 ]
In radiology teratomas appear round and often lobulated and contain
elements of soft tissue with fluid and fat, calcification seen in 20-43% of cases.
Seminoma, histology obtained approximately 40% of germ cell tumors.
Seminoma appears as a large mass is homogeneous whereas nonseminoma
heterogeneous mass with irregular edge caused by the invasion of the surrounding
tissue. To distinguish seminoma to nonseminoma used serum marker of beta-HCG
and alpha-fetoprotein. Approximately 10% of patients with pure seminoma menglami
increase in serum beta-HCG but there is no increase in serum alpha-fetoprotein.
Although the pure seminoma beta-HCG concentrations are sometimes high but not
high alphafetoprotein. While the concentrations of both markers nonseminoma was
always high. The concentration of beta-HCG and alpha-fetoprotein more than 500 mg
/ mL is a definite diagnosis for nonseminoma. Radiological picture, looks like a large
lobulated mass and homogeneous in the anterior mediastinum region. [ 5 , 6 ]
Below can be seen histological classification of germ cell tumors
Table 4. Classification of germ cell tumor histology

C. Neural Tumors
Nerve tumors occur approximately 20% in adults and 35% in children. These
tumors can grow on nerve cells disembarang places, more frequently in the posterior
mediastinum. Most are asymptomatic. The tumor can be benign or malignant and are
usually classified according to the network that formed it. Benign tumors are very
rarely become malignant. Although it is said often in children but can also be found
in adults. Topcu from Turkey analyzed 60 patients with tumors of nerves and get 13
people with babies and children age (<15 years), 47 adults (age> 15 years), more
women (39) than men (21). Only 20% (12 of 60) are malignant. [ 4 ]
Table 5. Histological classification of tumors of nerve
Tumors derived from peripheral blood cells usually asymptomatic lesions with
spherical morphology, treatment with excision. Meanwhile, a neuroblastoma tumor
cell derived neural chest. Neuroblastoma is an aggressive tumor and metastasize
quickly. This mass is not encapsulated and usually shows cystic degeneration,
hemorrhage, and necrosis. Most often occur in children. Clinical manifestations of
pain, neurological deficits, compressing the airway and ataxia. On CT scans, 80%
appear to calcification. Treatment with resection when the tumor is still being limited,
then resection enough, but if it has spread, it is necessary to chemotherapy and
radiation.
Table 6. Neuroblastoma Staging
Stage
Characteristics
I
Non-invasive, ipsilateral
II
Local invasion without extension. Ipsilateral lymph node involvement
III
Tumor spread and lymph node meewati midline bilaterally
IV
Metastasis
IVs
Stage I or II and metastasis to the liver, skin and bone marrow
D. Lymphoma
Lymphoma is the most common mass in the mediastinum around 50% among
all mediastinal masses. Tumor is located in the medial area and usually manifests as a
primary tumor. Hodgkin lymphoma is approximately 25-30% of cases of lymphoma.
Lymphoma is composed of a diverse group of neoplasms originating from malignant
proliferation of lymphocytes in the lymphoid system. Lymphoma can occur as a
result of genetic mutation or a viral infection. Produce malignant transformation of
cells with uncontrolled and excessive growth that accumulates in the lymph nodes so

as to form a mass. Lymphomas generally start in the lymph nodes and lymphoid
tissue in the stomach and intestines. [ 6 ]
Hodgkin lymphoma is a malignant lymphoma that occurs at a median age of
38 years. More common in whites than blacks. Characteristic is a progression from
one group of lymph nodes to other places and the appearance of systemic symptoms,
and the presence of Reed Sternbeg cell on histopathologic features. Patients with
Hodgin's lymphoma, usually found in cervical lymphadenopathy or supraclavikular.
30% is accompanied by fever, night sweats, or weight loss. Most mediastinal
lymphoma are asymptomatic and found incidentally on chest radiographs. It can also
be the symptom of chest pain, coughing, and wheezing sound or dysphagia due to the
mass invasion of mediastinal structures. Superior vena cava syndrome (SVCS) and
the invasion of the chest wall is rare. [ 6 , 12 ]
Non-Hodgkin lympoma including -cell neoplasms. The median age of
patients between 55-68 years older. Lymphadenopathy can occur locally and general.
Superior vena cava syndrome is common in these cases. The symptoms most
frequently found in the superior vena cava syndrome, coughing, hoarseness,
tightness, and chest pain. Biopsy is the primary diagnostic tool. [ 12 ]
Radiologically the photo posteroanterior chest x-ray showed
mediastinal widening medially and superiorly, the lateral thorax can be seen
retrosternal space. [ 8 ]
DIAGNOSIS
To perform diagnostic procedures mediastinal tumor to be seen if the
patient comes to the gravity. Most mediastinal tumor with no symptoms and is found
during chest X-ray done for a variety of reasons. Patients who come to the gravity of
breath often require emergency action. Patient complaints are usually related to the
size and invasion or compression of surrounding organs, such as severe shortness of
breath, superior vena cava syndrome (SVKS) and swallowing disorders. Not
infrequently the patient comes to the gravity of breath, cardiovascular or
gastrointestinal tract. When patients present with life-threatening severity, the
diagnostic procedures may be delayed. Meanwhile, therapy and measures to
overcome gravity, when it allows diagnostic procedure was performed. [ 4 , 12 ]
In general, the diagnosis of mediastinal tumor is established as follows:
1. Clinical Overview
Anamnesis
Mediastinal tumors often do not give symptoms and detected on chest Xray done. For benign tumors, complaints usually starts when there is an increase in
the size of the tumor that causes suppression of mediastinal structures, whereas
malignant tumors can cause symptoms due to compression or invasion of mediastinal
structures.
Symptoms and signs that may arise depending on the organ involved :
1.
Cough, shortness or stridor arise when compressing or invasion of
the trachea and / or main bronchi,
2.
Dysphagia caused by an emphasis or invasion of the esophagus
3.
Superior vena cava syndrome (SVKS) are more common in
mediastinal tumors were malignant compared with benign tumors,
4.
Hoarseness and dry cough appears when laringel involved nerve,
paralysis of the diaphragm phrenic nerve arises when emphasis
5.
Chest wall pain or neurogenic tumors arise in the nervous system
suppression.

 Physical examination
The physical examination will provide information in accordance with the
location, size and limitations of other organs, such as has occurred emphasis to
surrounding organs. The possibility of mediastinal tumors can be thought of or
associated with some clinical circumstances, for example :
1.
myasthenia gravis may indicate thymoma
2.
lymphadenopathy may indicate lymphoma
Laboratory studies (3, 4)
1.
The results of routine laboratory tests often do not provide
information relating to the tumor. LED sometimes increases in
lymphoma and tuberculosis.
2.
Examination of the levels of T3 and T4 is required for thyroid
tumors.
3.
Examination of -fetoprotein and -HCG performed for mediastinal
tumors including germ cell tumor group, that is, if there is any
doubt between seminoma or nonseminoma. Levels of -fetoprotein
and -HCG nonseminoma higher in group [ 4 , 13 ]
Radiology Procedures (3)
1.
CXR
Of CXR PA / lateral tumor location can be determined, the anterior,
medial or posterior, but in cases with large tumor size is difficult to
determine the exact location.
2.
Thoracic CT scan with contrast
Besides being able to decrypt the location of the tumor can also
decrypt disorder better and with the possibility to determine the
expected types of tumors, such as teratoma and thymoma. CT scan
can also determine the stage in the case of thymoma by finding
whether there has been an invasion or not. The development of this
tool facilitates the retrieval of materials for cytology. To determine
the extent of radiation several types of tumors of the mediastinum
should be performed a CT scan of thoracic and abdominal CTScan.
[ , ,
8 9 11 , 14 ]
3.
Flouroskopi
This procedure is done to see the possibility of aortic aneurysm.
4.
Esofagografi
This examination is recommended if there is suspicion of invasion
or emphasis to the esophagus.
5.
Ultrasound, MRI and Nuclear Medicine
Although rare, sometimes the tests should be done for some cases
of mediastinal tumor.
Endoscopic procedures [ 4 , 13 , 15 ]
1.
Bronchoscopy should be performed when indicated operation.
Action bronchoscopy can provide information about the
encouragement or suppression of tumors to respiratory and location.
In addition, via bronchoscopy can also be seen whether there was
tumor invasion into the airway. Bronchoscopy is often unable to
distinguish mediastinal tumors from primary lung cancer.
2.
Mediastinokopi. This action is preferred for tumors located in the
anterior mediastinum.
3.
Esofagoskopi

 Procedures Pathology [ 4 , 16 ]
1. Cytology
Diagnostic procedures to obtain material for cytology examination are:
biopsy, fine needle (fine needle aspiration BJH or biopsy / FNAB),
performed when enlarged superficial lymph node or tumor.
a. pleural puncture if there is pleural effusion
b. washings or bronchial brushings during bronchoscopy
c. needle aspiration biopsy, which is taking a material with a needle that is
done when the future looks intrabronkial during bronchoscopy
procedures are very easy to bleed, so the biopsy is very dangerous
transthoracal transthoracic biopsy or biopsy (TTB) is done when the mass
can be achieved with a needle that is inserted in the chest wall and not near
the location of the tumor blood vessel aneurysm or no suspicion. For small
tumors (<3cm>, have many blood vessels and nearby organs at risk can be
done with the guidance flouroskopi TTB or guiding ultrasound or CT scan.
This method provides a high diagnostic yield and are not influenced by the
size and location of the tumor. [ 4 , 13 , 16 ]
2. Histologic examination [ 4 , 16 , 17 ]
When BJH not been able to establish histologic type, needs to be carried
out the following procedure:
a. biopsy of palpable lymph nodes in the neck or supraclavicular. If there
is no palpable lymph nodes, network nodes can be done lifting that
might be there. This procedure is called a biopsy Daniels.
b. mediastinal biopsy, performed when the above actions have not
obtained results. Tao tahin FW et al in 2007 reported that the anterior
mediastinal tumor area for diagnostic histology can be done is to do a
mini mediastinotomi making a small incision of approximately 3 cm
area parasternalis line intercostal spaces 2 or 3. Mini mediastinotomi
This is a method that is safe, minimally invasive, reasonably priced and
give satisfactory results.
c. excisional biopsy of the tumor mass is large
d. diagnostic thoracoscopic
e. Video-assisted thoracic surgery (VATS), performed for tumors in all
locations, especially in the posterior part of the tumor.
3. Measures Surgery
Exploratory thoracotomy for diagnostic if all diagnostic efforts
4. Inspection Other
EMG is the investigation of mediastinal tumor or tumor type thymoma
tumorvlainnya. The usefulness of this examination is exploring the
possibility of miestenia gravis or myesthenic reaction.
MANAGEMENT
The management of mediastinal tumors depend on the nature of the tumor,
benign or malignant. Actions for mediastinal tumors that are benign are surgical,
while malignant tumors by type. Malignant mediastinal tumor types most commonly
found are thymoma, lymphoma, germ cell tumor and nerve.
In general, therapy for malignant tumors of the mediastinum is
multimodaliti ie surgery, chemotherapy and radiation. Some types of tumors are
resistant to radiation and / or chemotherapy to surgery becomes the treatment of
choice, but many other types should get multimodaliti action. Chemoradiotherapy

may be given before surgery (neoadjuvant) or after surgery (adjuvant). Therapeutic

options for thymoma is determined by staging the disease at diagnosis. For germ cell
tumors depend on tumor subtypes whereas nerve tumors based on tumor tissue
dominant. [ 4 ]
1997 data from the Department of Radiology University of Pittsburgh:
neurogenic tumors, thymoma, and approximately 60% of benign cysts can be
resected compared limphoma, teratoma and granulomatous about 30% that can be
resected. Approximately 2/3 of all mediastinal tumors are benign tumors. More than
75% of patients had no complaints is a benign tumor lesion, while almost two thirds
of patients with symptoms of a malignant mediastinal mass lesions. [ 4 ]
Management of mediastinal tumor

Flow management nonseminoma germ cell tumors

Management of germ cell tumors

CONCLUSION
Mediastinal tumor is a tumor that is contained within the mediastinal cavity is
located between the right and left lung
Mediastinal cavity is narrow and can not be expanded, enlarged tumors can press
on nearby organs and can cause life-threatening severity
Mediastinal tumors are asymptomatic and discovered a lot at the time of chest Xray for a variety of reasons.
Complaints in patients with mediastinal tumors are usually associated with the size
and the compression of the surrounding organs such as severe shortness of
breath, superior vena cava syndrome and swallowing disorders
Differences in lung tumors and mediastinal tumors: a mass in the mediastinum will
not have water bronchogram, lung mass was forming a sharp angle
mediastinal mass forms an obtuse angle.
The management of mediastinal tumors are very dependent on the tumor is benign
or ferociously. Benign tumors surgical intervention, whereas for malignant
tumors depending on the species, but in general is multimodaliti therapy is
surgery, chemotherapy and radiation

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