M ediastinaltum ors

Yani Purnamasari
Sp.P

 Mediastinal tumors are benign or cancerous

growths that form in the area of the chest that

separates the lungs.
the mediastinum, is surrounded by the

breastbone in front, the spine in back, and the

lungs on each side (lower: diafragma, lateral:
pleura mediastinal)
The mediastinum contains the heart, aorta,

esophagus, thymus and trachea.

The m ediastinum is divided into 3 sections:

The anterior (front)
The middle
The posterior (back)

Mediastinum tumors are mostly made

of :
reproductive (germ) cells or develop
in thymic,
neurogenic (nerve),
lymphatic or mesenchymal (soft)
tissue.

W ho is af e
fcted by m ediastinaltum ors?
In general, mediastinal tumors are rare. Mediastinal

tumors are usually diagnosed in patients aged 30 to 50

years, but they can develop at any age and form from any
tissue that exists in or passes through the chest cavity.
The location of tumors within the mediastinum varies

according to the age of the patient.

In children, tumors are commonly found in the posterior

(back) mediastinum. These mediastinal tumors often begin

in the nerves and are typically benign (noncancerous).
In adults, most mediastinal tumors occur in the anterior

(front) mediastinum and are generally malignant

(cancerous) lymphomas or thymomas.

Is a m ediastinal tum or
seri
Dueous?
to their location in the mediastinum
mediastinal tumors (both benign and
malignant) serious complications
including invading the heart, pericardium
(the lining around the heart), and great
vessels (the aorta and vena cava).
Tumors located in the posterior (back)

mediastinum can cause compression of

the spinal cord.

There are severaltypes ofm ediastinaltum ors

Anterior (front) mediastinum
Germ cell - The majority of germ cell neoplasms (60 - 70%)

are benign and are found in both males and females.

Lymphoma Malignant tumors

( Hodgkins disease and

non Hodgkins lymphoma).

Thymoma and thymic cyst - a thymic mass, the majority

of thymomas are benign lesions ( contained within a fibrous

capsule). About 30% of these more aggressive and
become invasive through the fibrous capsule.
Thyroid mass mediastinal Usually a benign growth,

such as a goiter, these can occasionally be cancerous.

M iddle m ediastinum
Bronchogenic cyst A benign growth with respiratory origins.
Lymphadenopathy mediastinal An enlargement of the lymph

nodes.
Pericardial cyst A benign growth that results from an "out-pouching"

of the pericardium (the hearts lining).

Thyroid mass mediastinal Usually a benign growth, such as a

goiter. These types of tumors can occasionally be cancerous.

Tracheal tumors These include tracheal neoplasms and non-

euplastic masses, such as tracheobronchopathia osteochondroplastica

(benign tumors).
Vascular abnormalities including aortic aneurysm and aortic dissection.

P osterior (back) m ediastinum

Extramedullary haematopoiesis A rare cause of

masses that form from bone marrow expansion and are

associated with severe anemia.
Lymphadenopathy mediastinal An enlargement of the

lymph nodes.
Neuroenteric cyst mediastinal A rare growth, which

involves both neural and gastrointestinal elements.

Neurogenic neoplasm mediastinal The most common

cause of posterior mediastinal tumors, these are classified

as nerve sheath neoplasms, ganglion cell neoplasms, and
paraganglionic cell neoplasms. ( 70% of neurogenic
neoplasms are benign )

W hat are the sym ptom s of a

m ediastinaltum or?
Cough
Shortness of breath
Chest pain
Fever
Chills
Night sweats
Coughing up blood
Hoarseness
Unexplained weight loss
Lymphadenopathy (swollen or tender lymph nodes)
Wheezing
Stridor (a high-pitched, noisy respiration, which can be a sign

of respiratory obstruction, especially in the trachea or larynx)

The symptoms mediastinal tumor caused of

tumor invasion , such as :
N. Phrenicus diafragma paralysis
N. reccurent Vocalis Cord paralysis
NN. Simpaticus Horner syndroma
( APEM )
Esophageus disphagia
V. Cava Sup venous congestion ( VCSS )
Trachea / Bronchus atelectasis
Cor pericard efusion

H ow is a m ediastinaltum or diagnosed?
Chest x-ray
Computed tomography (CT) scan of the chest or CT-guided

needle biopsy
Magnetic resonance imaging (MRI) of the chest
Mediastinoscopy with biopsy (Performed under general

anesthesia) this examination of the chest cavity uses a

lighted tube inserted through a small incision under the
chest bone; a sample of tissue is taken to determine if
cancer is present. Mediastinoscopy ( accurately diagnose
80 - 90% of mediastinal tumors, and 95 - 100% of anterior
mediastinal tumors.)

H ow are m ediastinal tum ors

treated?
Depends on the type of tumor and its location:
Thymic cancers require surgery, followed by radiation

or chemotherapy. Types of surgery include

thoracoscopy , mediastinoscopy and thoracotomy (a
procedure performed through an incision in the chest).
Lymphomas are recommended to be treated with

chemotherapy followed by radiation.

Neurogenic tumors found in the posterior (back)

mediastinum are treated surgically.

M ediastinaltum ors
Tumors of the mediastinum represent a wide diversity

of disease states
The most common causes of an anterior mediastinal

mass include: thymoma, germ cell tumor, thyroid

disease and lymphoma
Masses of the middle mediastinum are typically

congenital cysts and lymphoma

In the posterior mediastinum are often neurogenic

tumors ,esophageal tumors and tumors of spinal

column

Thym om as
The most important determinants of
long term survival in thymoma are :
complete resection
Masaoka stage
WHO histologic classification
Strobel et al. Tumor recurrence and survival in
patients treated for thymomas and thymic Sq cell
carcinomas; A retrospective analysis. J Clin Oncol
2004 ;22;1501-1509

Thym om as
WHO classification of thymomas
WHO type Cytological Features
Type A Medullary, spindle cell
Type AB Mixed Thymomas
Type B1 Organoid, predominantly cortical , lymphocyte

predominant
Type B2 Cortical
Type B3 Well diff Ca , epithelial predominant , squamoid
Type C Thymic carcinoma
From Wilkins et al. Cases of Thymoma of the Massachusetts General Hospital .
J Thoracic Cardiovascular Surg 1966, 52; 322-330

M asaoka Staging System for Thym om a

Stage Description
I Macroscopically completely encapsulated with no microscopic

detectable capsular invasion

II Macroscopic invasion into surrounding mediastinal fatty tissue or

mediastinal pleura or microscopic invasion in the capsule

III

Macroscopic invasion into surrounding organs or intrathoracic

metastases or both (pericardium, great vessels, heart)

IV A Pleural or pericardial implants/dissemination

IVB Lymphagenous or hematogenous metastases

Adapted from Masaoka et al. Follow up study of thymomas with special

reference to their clinical stages .Cancer 1981 ;48; 2485,with permission

Thym om as
Several studies have attempted to correlate morphologic

staging with tumor invasion and prognosis

It appears that medullary ( WHO A) and mixed (WHO AB) are

not invasive and typically correspond to Masaoka stg I and II

Cortical thymomas (WHO B1,2 and 3)are more invasive and

occur more commonly as stg III and IV lesions

The Masaoka stg system remains the most widely accepted

stg upon which current management recommendations are

based
Shneidar PM. et al. Prognostic importance of histomorpohologic sub classification for epithelial
thymic tumors . Ann Surg Oncol 1996;4;46-56
Nakagawa et al. Thymoma ; Clinicopathological study based on the new WHO classification ; J
Thora

Thym om as
Influence of Masaoka stg on complete resection,

recurrence and survival (n=1320)

Masaoka stage
Complete
resection(%)
Recurrence (%)
5-Year survival(%)

II

III

IVA

100

100

85

42

28

34

100

98

89

71

Data from Kondo et al.Ann Thorac.Surg.2003;76:878

85(35)

Treatm ent ofTym om a

Stage I : Extended thymo thymecthomy

(ETT)
Stage II :ETT, radiation
Stage III : ETT & extended resection,
radiotherapy & chemotherapy
Stage IVA: Debulking,chemotherapy &
radiotherapy
Stage IVB: chemotherapy &
radiotherapy ,
debulking

Treatm ent Thym ic carcinom a

The same multy-modality on lung

carcinoma

Treament Thymic carcinoid &

oatcell carcinoma
Surgery, radiotherapy & chemotherapy
post surgery (because invasive tumor)

 The myasthenia gravis /myasthenic

reaction Plasmapharesis before

surgery (fastest one week before
surgery)
The symptom myasthenic reaction
before surgery myasthenia
gravis treatment

M ultim odality treatm ent oflocally

advanced thym om as
Potentially operable stage III disease are best treated with

induction therapy follow by surgery

The combination of PAC( cisplatin, doxorubicin,

cyclophosphomide) or ADOC (cisplatin, doxorubicine, vincristine,

cyclophosphomide) have reported objective response rates from
77-100% with resectability rates ranging between 40- 70%
The administration of neoadjuvant chemotherapy improved 10

yr survival from 71% (no induction therapy) to 90%.

Lucchi M ,et al. Advanced stage thymomas and thymic carcinomas : results of
multimodality treatments. Ann Thorac Surg 2005 ;79 ;1840-4
Venuta f, et al. Long term outcome after multimodality treatment for stage III thymic
tumors. Ann Thorac Surg 2003;76:1866-72

G erm celltum or ofthe

m ediastinum
Histologic classification :
Seminoma
Non seminoma
Embryonal carcinoma
Choriocarcinoma
Yolksac carcinoma

Teratoma
Benigna
Malignant ( germinal cell, nongerminal,

immature)

Sem inom a Treatm ent

Chemoradio therapy sensitif
No surgery indication

Nonseminoma treatment :
Radioresisten & chemotherapy 6
cycle
Evaluation on 3-4 cycle with -HCG
& -fetoprotein, thoracs photo PA &
lateral

Benigna Teratom a treatm ent

surgery

Malignan Teratoma :
Multimodality
(surgery,chemotherapy,radiotherapy

The important thinks about

malignanTeratoma :
T.Mature on adult not always
benigna
T.immature on child not always
malignan
T.mature on child always benigna

N eurogenic Tum or
Histologic classification :
Peripheral nerves
Neurofibroma
Neurilemoma (schwannoma)
Neurosarcoma

Symphathetic ganglia
Ganglioneroma
Ganglioneuroblastoma
Neuroblastoma

Paraganglionic
Pheochromacythoma
Paraganglioma

Treatm ent ofneurogenic tum or

Surgery,
Except neuroblastoma (radiosensitif)

radiochemotherapy
Neurilemoma (Schwanoma)
chemotherapy adjuvant (prevent
reccurency)

M esenchim altum or & Endocrine tum or

Rare found
Specific treatment

Evaluation oftreatm ent m ediastinal

tum ors
Side efec chemotherapy
Respons of therapy after 2 cycle or

radiation 2000 cGy & photo thoracs

Partial respons / stable disease
chemotherapy &radiation
Stop treatment if progresive diseases

Thank You

Prosedur D iagnostik tum or m ediastinum tanpa

kegaw atan
Tumor mediastinum tanpa kegawatan (telah
teratasi)
Anamnesis,Px. Fisik, lab.
rutin
Radiologik

Endosko
pi

Foto
toraks
PA/lateral
Tomografi
CT Scan
toraks dg
kontras
Px. lain
Fluoroskop
i,Esofagog
rafi,Angiog
rafi, USG,
Kedoktera
n Nuklir,
MRI

Bronkosko
pi,
Mediastino
skopi,
Esofagosko
pi,
Torakoskop
i

Histopatolo
gi

Laboratori
um

Sitologi,B
JH/FNAB,
punksi
Pleura,
Bilasan/si
katan/BA
L
bronkus

Histologi:
Biopsi
KGB/Dani
el, Biopsi
Mediastin
al, Biopsi
Eksisiona
l

TTB, TTB
CTGuide

Torakosk
opi,
VATS,Bio
psi lain

Torakotom
i
eksplorasi

-Fetoprotein,
-HCG

Prosedur diagnostik tum or m ediastinum dg kegaw atan

Tumor Mediastinum dg
kegawatan
Kegawatan non
napas
K.kardiovascul
K.sal.cer
er
na
SVKS
Peneka
Beda
nan
h
aorta
Pros
edur
dpt
dilak
ukan
Prose
dur
diagn
ostik

Prosedur
tdk dpt
dilakukan
Radiasi
300-1000
cGy
Prosedur
diagnost
ik
lanjutka

Beda
h

Kegawatan
napas
Steroid + Radiasi 3001000 cGy + stand by
bedah
Respons
(+)
(klinis
membai
k)
Prosedur
diagnostik
dilanjutka
n

Respons
(-) (msh
ada
gangguan
)
Beda
h

ECC
(Extra
Corpored
Circulation

Tanpa
ECC

Penatalaksanaan Tum or m ediastinum

Tumor
mediastinum
Jina
k
Beda
h

Gana
s
Limfom
a

Hodgkin
s
Penatalaksa
naan sesuai
dg Limfoma
Hodgkins

Non
Hodgkin
s
Penatalaksa
naan sesuai
dg Limfoma
Non
Hodgkins

Non
limfoma
Timom
a&
Tumor
Timus

Germ
cell

Tumo
r
neuro
genik

Tumor
mesengi
al &
Tumor
endokrin

Penatalaksanaan Tum or SelG erm inalN onsem inom a

m ediastinum
Kemoterapi 3-4
siklus
Kadar HCG
&AFP
normal,foto
toraks
normal
Tidak perlu terapi
Teratoma
jinak &
jar.nekrotik

Tidak perlu
terapi
lanjutan

Kadar HCG & AFP

normal, foto
toraks
stabil/abnormal

Lanjutan
kemoterapi

Reseksi
bedah
Tumor dpt
diangkat /
ada sisa
tumor
Lanjutka
n
kemotera
pi

Kadar HCG
atau AFP
meningkat

Kadar HCG &AFP

normal, foto
toraks
stabil/abnormal

Kadar
HCG &
AFP tetap
tinggi
Suportive
care

K lasifi
kasiTum or m ediastinum m enurut
R osen berg

Neurogenic

Germ cell Tumor

Endocrine
Tumor

Arising from peripheral

nerves

Seminoma

Thyroid

Neurofibroma

Nonseminomatous
tumors

Thyroid

Neurilemoma
(Schwanoma)

Pure embryonal cell

Parathyroid

Neurosarcoma

Mixed embryonal cell

Cysts

Arising from
symphatetic ganglia

With seminomatous
elements

Pericardial

Ganglioneuroma

With trophoblastic
elements

Bronchogenic

Ganglioneuroblastoma

With teratoid elements

Enteric

Neuroblastoma

With entodermal sinus

elements

Thymic

Arising from
paraganglionic tissue

Teratoma,benign

Thoracic duct

Pheochromocytoma

Meningoceles

Thymic

Aneurysms

Hernias

Thymoma

Mesenchymal Tumors

Hiatal

Carcinoid

Fibroma & Fibrosarcoma

Morgagni

Thymolipoma

Lipoma & Liposarcoma

Myxoma
Mesothelioma
Leiomyoma &
Leiomyosarcoma

Lymphoma

Rhabdomyosarcoma

Lymphadenopat
hy

Hodgkins disease

Xantogranuloma

Inflamatory

Histiocytic
Lymphoma

Mesenchymoma

Granulomatous

Undifferentiated

Hemangioma

Sarcoid

Hemangioendothelioma
Hemangiopericytoma
Lymphangioma
Lymphangiomyoma