week 4 meadiastinumtumor- 20 november 2012

8/17/2019 Week 4 MeadiastinumTumor- 20 November 2012

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M ediastinal tum ors

Yani PurnamasariSp.P


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Mediastinal tumors are benign or cancerousgrowths that form in the area of the chest thatseparates the lungs.

the mediastinum, is surrounded by thebreastbone in front, the spine in back, and thelungs on each side (lower: diafragma, lateral:

pleura mediastinal)

he mediastinum contains the heart, aorta,esophagus, thymus and trachea.


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The m ediastinum is divided into 3 sections:

he anterior (front)

he middle he posterior (back)


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Mediastinum tumors are mostly madeof :

reproducti!e (germ) cells or de!elopin thymic,

neurogenic (ner!e),

lymphatic or mesenchymal (soft)tissue.


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W ho is affected by m ediastinal tum ors?

"n general, mediastinal tumors are rare. Mediastinaltumors are usually diagnosed in patients aged #$ to %$years, but they can de!elop at any age and form from anytissue that e&ists in or passes through the chest ca!ity.

he location of tumors within the mediastinum !ariesaccording to the age of the patient.

"n children, tumors are commonly found in the posterior(back) mediastinum. hese mediastinal tumors often begin

in the ner!es and are typically benign (noncancerous).

"n adults, most mediastinal tumors occur in the anterior(front) mediastinum and are generally malignant(cancerous) lymphomas or thymomas.


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Is a m ediastinal tum or

serious? 'ue to their location in the mediastinum mediastinal tumors (both benign andmalignant) serious complicationsincluding in!ading the heart, pericardium

(the lining around the heart), and great!essels (the aorta and !ena ca!a).

umors located in the posterior (back)mediastinum can cause compression ofthe spinal cord.


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There are several types of m ediastinal tum ors

Anterior (front) mediastinum

Germ cell * he ma+ority of germ cell neoplasms ($ * -$)are benign and are found in both males and females.

Lymphoma / Malignant tumors ( 0odgkin1s disease andnon 0odgkin1s lymphoma).

Thymoma and thymic cyst * a thymic mass, the ma+orityof thymomas are benign lesions ( contained within a 2brouscapsule). 3bout #$ of these more aggressi!e and

become in!asi!e through the 2brous capsule.

Thyroid mass mediastinal / 4sually a benign growth,such as a goiter, these can occasionally be cancerous.


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M iddle m ediastinum

Bronchogenic cyst / 3 benign growth with respiratory origins.

Lymphadenopathy mediastinal / 3n enlargement of the lymphnodes.

Pericardial cyst / 3 benign growth that results from an 5out*pouching5of the pericardium (the heart1s lining).

Thyroid mass mediastinal / 4sually a benign growth, such as agoiter. hese types of tumors can occasionally be cancerous.

Tracheal tumors / hese include tracheal neoplasms and non*euplastic masses, such as tracheobronchopathia osteochondroplastica(benign tumors).

6ascular abnormalities including aortic aneurysm and aortic dissection.


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Posterior (back) m ediastinum

Extramedullary haematopoiesis / 3 rare cause ofmasses that form from bone marrow e&pansion and areassociated with se!ere anemia.

Lymphadenopathy mediastinal / 3n enlargement of thelymph nodes.

Neuroenteric cyst mediastinal / 3 rare growth, whichin!ol!es both neural and gastrointestinal elements.

Neurogenic neoplasm mediastinal / he most commoncause of posterior mediastinal tumors, these are classi2edas ner!e sheath neoplasms, ganglion cell neoplasms, andparaganglionic cell neoplasms. ( -$ of neurogenicneoplasms are benign )


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W hat are the sym ptom s of am ediastinal tum or?

7ough

Shortness of breath

7hest pain

8e!er

7hills

9ight sweats

7oughing up blood

0oarseness

4ne&plained weight loss

ymphadenopathy (swollen or tender lymph nodes)

;hee


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he symptoms mediastinal tumor caused of

tumor in!asion , such as : / 9. Phrenicus diafragma paralysis

/ 9. reccurent 6ocalis 7ord paralysis

/ 99. Simpaticus 0orner syndroma( 3P=M )

/ =sophageus disphagia

/ 6. 7a!a Sup !enous congestion ( 67SS )

/ rachea > ?ronchus atelectasis / 7or pericard efusion


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H ow is a m ediastinal tum or diagnosed?

7hest &*ray

7omputed tomography (7) scan of the chest or 7*guidedneedle biopsy

Magnetic resonance imaging (M@") of the chest

Mediastinoscopy with biopsy (Performed under generalanesthesia) this e&amination of the chest ca!ity uses alighted tube inserted through a small incision under thechest boneA a sample of tissue is taken to determine ifcancer is present. Mediastinoscopy ( accurately diagnoseB$ * C$ of mediastinal tumors, and C% * D$$ of anteriormediastinal tumors.)


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H ow are m ediastinal tum orstreated?'epends on the type of tumor and its location:

hymic cancers reEuire surgery, followed by radiation or chemotherapy. ypes of surgery includethoracoscopy , mediastinoscopy and thoracotomy (a

procedure performed through an incision in the chest).

ymphomas are recommended to be treated withchemotherapy followed by radiation.

9eurogenic tumors found in the posterior (back)mediastinum are treated surgically.


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M ediastinal tum ors

umors of the mediastinum represent a wide di!ersityof disease states

he most common causes of an anterior mediastinalmass include: thymoma, germ cell tumor, thyroiddisease and lymphoma

Masses of the middle mediastinum are typically

congenital cysts and lymphoma

"n the posterior mediastinum are often neurogenictumors ,esophageal tumors and tumors of spinalcolumn


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Thym om as

he most important determinants oflong term sur!i!al in thymoma are :

complete resection Masaoka stage

;0F histologic classi2cation

Strobel et al. umor recurrence and sur!i!al inpatients treated for thymomas and thymic SE cellcarcinomasA 3 retrospecti!e analysis. G 7lin FncolH$$I AHHAD%$D*D%$C


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Thym om as

;0F classi2cation of thymomas

;0F type 7ytological 8eatures

ype 3 Medullary, spindle cell

ype 3? Mi&ed hymomas

ype ?D Frganoid, predominantly cortical , lymphocytepredominant

ype ?H 7ortical

ype ?# ;ell diJ 7a , epithelial predominant , sEuamoid ype 7 hymic carcinoma

8rom ;ilkins et al. 7ases of hymoma of the Massachusetts Keneral 0ospital . G horacic 7ardio!ascular Surg DC, %HA #HH*##$


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M asaoka Staging System for Thym om a

Stage 'escription

" Macroscopically completely encapsulated with no microscopicdetectable capsular in!asion

"" Macroscopic in!asion into surrounding mediastinal fatty tissue or

mediastinal pleura or microscopic in!asion in the capsule

""" Macroscopic in!asion into surrounding organs or intrathoracicmetastases or both (pericardium, great !essels, heart)

"6 3 Pleural or pericardial implants>dissemination

"6? ymphagenous or hematogenous metastases

3dapted from Masaoka et al. 8ollow up study of thymomas with specialreference to their clinical stages .7ancer DCBD AIBA HIB%,with permission


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Thym om as Se!eral studies ha!e attempted to correlate morphologic

staging with tumor in!asion and prognosis

"t appears that medullary ( ;0F 3) and mi&ed (;0F 3?) arenot in!asi!e and typically correspond to Masaoka stg " and ""

7ortical thymomas (;0F ?D,H and #)are more in!asi!e andoccur more commonly as stg """ and "6 lesions

he Masaoka stg system remains the most widely accepted

stg upon which current management recommendations arebased

Shneidar PM. et al. Prognostic importance of histomorpohologic sub classi2cation for epithelialthymic tumors . 3nn Surg Fncol DCCAIAI*%

9akagawa et al. hymoma A 7linicopathological study based on the new ;0F classi2cation A G

hora


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Thym om as

"nLuence of Masaoka stg on complete resection,recurrence and sur!i!al (nD#H$)

Masaoa stage ! !! !!! !"A

7ompleteresection()

D$$ D$$ B% IH

@ecurrence () D I HB #I

%*Year sur!i!al() D$$ CB BC -D

'ata from Nondo et al.3nn horac.Surg.H$$#A-:B-B

B%(#%)


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Treatm ent of Tym om a

Stage ": =&tended thymo thymecthomy(=)

Stage "" :=, radiation Stage """ : = O e&tended resection,

radiotherapy O chemotherapy

Stage "63: 'ebulking,chemotherapy Oradiotherapy

Stage "6?: chemotherapy Oradiotherapy , debulking


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Treatm ent Thym ic carcinom a

he same multy*modality on lungcarcinoma

reament hymic carcinoid Ooatcell carcinoma

• Surgery, radiotherapy O chemotherapypost surgery (because in!asi!e tumor)


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he myasthenia gra!is >myasthenicreaction Plasmapharesis beforesurgery (fastest one week beforesurgery)

he symptom myasthenic reactionbefore surgery myasthenia

gra!is treatment


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M ultim odality treatm ent of locallyadvanced thym om as

Potentially operable stage """ disease are best treated withinduction therapy follow by surgery

he combination of P37( cisplatin, do&orubicin,

cyclophosphomide) or 3'F7 (cisplatin, do&orubicine, !incristine,cyclophosphomide) ha!e reported ob+ecti!e response rates from--*D$$ with resectability rates ranging between I$* -$

he administration of neoad+u!ant chemotherapy impro!ed D$yr sur!i!al from -D (no induction therapy) to C$.

ucchi M ,et al. 3d!anced stage thymomas and thymic carcinomas : results ofmultimodality treatments. 3nn horac Surg H$$% A-C ADBI$*I

6enuta f, et al. ong term outcome after multimodality treatment for stage """ thymictumors. 3nn horac Surg H$$#A-:DB*-H


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Germ cell tum or of the

m ediastinum0istologic classi2cation : Seminoma

9on seminoma =mbryonal carcinoma

7horiocarcinoma

Yolksac carcinoma

eratoma ?enigna

Malignant ( germinal cell, nongerminal,

immature)


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Sem inom a Treatm ent

7hemoradio therapy sensitif 9o surgery indication

9onseminoma treatment :@adioresisten O chemotherapy cycle=!aluation on #*I cycle with *07K

O Q*fetoprotein, thoracs photo P3 Olateral


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Benigna Teratom a treatm ent surgery

Malignan eratoma :Multimodality

(surgery,chemotherapy,radiotherapy he important thinks aboutmalignaneratoma :

• .Mature on adult not alwaysbenigna• .immature on child not alwaysmalignan• .mature on child alwa s beni na


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Neurogenic Tum or

0istologic classi2cation : Peripheral ner!es 9euro2broma

9eurilemoma (schwannoma)

9eurosarcoma

Symphathetic ganglia Kanglioneroma

Kanglioneuroblastoma

9euroblastoma

Paraganglionic Pheochromacythoma

Paraganglioma


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Treatm ent of neurogenic tum or

Surgery,

=&cept neuroblastoma (radiosensitif)

radiochemotherapy 9eurilemoma (Schwanoma)

chemotherapy ad+u!ant (pre!entreccurency)


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M esenchim al tum or & Endocrine tum or

@are found

Speci2c treatment


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Evaluation of treatm ent m ediastinaltum ors

Side efec chemotherapy

@espons of therapy after H cycle or

radiation H$$$ cKy O photo thoracs Partial respons > stable disease

chemotherapy Oradiation

Stop treatment if progresi!e diseases


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Thank ou


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Prosedur D iagnostik tum or m ediastinum tanpakegaw atan

umor mediastinum tanpa kegawatan (telahteratasi)

3namnesis,P&. 8isik, lab.rutin

@adiologik =ndoskopi

0istopatologi

aboratorium

orakotomieksplorasi8oto

toraksP3>lateral omogra27 Scantoraks dgkontras

P&. lain8luoroskopi,=sofagogra2,3ngiogra2, 4SK,Nedoktera

n 9uklir,M@"

?ronkosko

pi,Mediastinoskopi,=sofagoskopi,

orakoskop

i

Sitologi,?

G0>893?,punksiPleura,?ilasan>sikatan>?3

bronkus

?, ?7*Kuide

0istologi:

?iopsiNK?>'aniel, ?iopsiMediastinal, ?iopsi=ksisiona

l

orakoskopi,63S,?iopsi lain

α-Fetoprotein,

β-HCG


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Prosedur diagnostik tum or m ediastinum dg kegaw atan

umor Mediastinum dgkegawatan

Negawatan nonnapas

Negawatannapas

N.kardio!asculer

N.sal.cerna

Steroid R @adiasi #$$*D$$$ cKy R stand bybedahS6NS Peneka

nanaorta

?eda

h

Prosedurdptdilakukan

Prosedurdiagn

ostik

Prosedurtdk dptdilakukan

@adiasi#$$*D$$$cKyProsedurdiagnost

iklan+utka

?edah

@espons(R)(klinismembaik)

@espons(*) (mshadagangguan)

Prosedur

diagnostikdilan+utkan

?edah

=77(=&tra7orpored

7irculation

anpa=77


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!enatalaksanaan Tum or m ediastinum

umormediastinum

Ginak

Kanas

?edah

imfoma

9onlimfoma

0odgkin1s

9on0odgkin1s

Penatalaksanaan sesuai

dg imfoma0odgkin1s

Penatalaksanaan sesuai

dg imfoma9on

0odgkin1s

imoma O

umor imus

Kermcell

umorneurogenik

umormesengial O

umorendokrin


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Penatalaksanaan um or !el "erm inal # onsem inom am ediastinum

Nemoterapi #*Isiklus

Nadar 07KO38Pnormal,fototoraks

normal

Nadar 07K O 38Pnormal, fototoraksstabil>abnormal

Nadar 07Katau 38Pmeningkat

idak perlu terapi@eseksibedah

an+utankemoterapi

eratoma +inak O

+ar.nekrotik

umor dptdiangkat >

ada sisatumor

Nadar 07K O38Pnormal, foto

toraksstabil>abnormal

Nadar07K O

38P tetaptinggi

idak perluterapilan+utan

an+utkankemoterapi

Suporti!ecare


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$lasifikasium orm ediastinum m enurut


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$lasifikasi um or m ediastinum m enurut%osen"erg

Neurogenic Germ cell Tumor EndocrineTumor

3rising from peripheral

ner!es

Seminoma hyroid

9euro2broma 9onseminomatoustumors

hyroid

9eurilemoma(Schwanoma)

Pure embryonal cell Parathyroid

9eurosarcoma Mi&ed embryonal cell 7ysts

3rising fromsymphatetic ganglia

;ith seminomatouselements

Pericardial

Kanglioneuroma ;ith trophoblasticelements

?ronchogenic

Kanglioneuroblastoma ;ith teratoid elements =nteric

9euroblastoma ;ith entodermal sinuselements

hymic

3rising fromparaganglionic tissue

eratoma,benign horacic duct

Pheochromocytoma Meningoceles

Th i A # i


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Thymic Aneurysms #ernias

hymoma Mesenchymal umors 0iatal

7arcinoid 8ibroma O 8ibrosarcoma Morgagni

hymolipoma ipoma O iposarcoma

My&oma

Mesothelioma

eiomyoma Oeiomyosarcoma

ymphoma @habdomyosarcoma ymphadenopathy

0odgkin1s disease antogranuloma "nLamatory

0istiocyticymphoma

Mesenchymoma Kranulomatous

4ndiJerentiated 0emangioma Sarcoid

0emangioendothelioma

0emangiopericytoma

ymphangioma

week 4 meadiastinumtumor- 20 november 2012

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