PEDIATRIC SOLID TUMORS
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Transcript of PEDIATRIC SOLID TUMORS
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PEDIATRIC SOLID TUMORS
Prof. Dr. Rejin KEBUDİ
I.U., Cerrahpaşa M. F., Dept. Of Pediatrics &
I.U., Oncology Institute, Div.Pediatric Hematology-Oncology
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Time (Months)
Su
rviv
al (
%) 65.8%
T.Kutluk & A. Yesilipek, on behalf of TPOG/TPHD
Turkish Pediatric Oncology Group (TPOG) & Turkish Pediatric Hematolgy Society (TPHD)
Pediatric Tumor Registry, 2002-2008Survival Rate (7 Year)
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Time (Months)
Su
rviv
al (
%)
T.Kutluk & A. Yesilipek, on behalf of TPOG/TPHD
Turkish Pediatric Oncology Group (TPOG) & Turkish Pediatric Hematolgy Society (TPHD)
Pediatric Tumor Registry, 2002-2008Survival rates in different tumor types
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Cancer In Children
Cancer Types % Acute Leukemia 28%
CNS Tumors 21%
Lymphomas 11%
Neuroblastoma 7.5%
Wilms Tumor 6%
Soft Tissue Sarcomas 6%
Bone Sarcomas 5%
Retinoblastoma 3%
Other 12.5%
Rhabdomyosarcoma
Non-RMS STS
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Çocukluk çağı kanserlerinin dağılımı
Beyin Tm19%
Lenfoma13%
Nöroblastom8%
Yumuşak Doku Tm8%
Wilms Tm6%
Kemik Tm5%
Retinoblastom3%
Diğer Tm8%
Lösemi30%
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T.Kutluk & A. Yesilipek, on behalf of TPOG/TPHD
100,00Total 8355
1,087
2,7226
4,4371
6,1509
1,5122
5,6470
2,3193
6,0505
7,4622
13,01084
18,61552
31,32614
%Tumor Type
n
Turkish Pediatric Oncology Group (TPOG) & Turkish Pediatric Hematology Society (TPHD)
Pediatric Tumor Registry, 2002-2008
XII Other and Unspecified Malignant Neoplasm
XI Carcinomas and Other Malignant Epithelial Neoplasm
X Germ Cell, Trophoblastic and Other Gonadal Neoplasm
VIII Malignant Bone Tumors
VII Hepatic Tumors
VI Renal Tumors
V Retinoblastoma
IX Soft-TIissue Sarcomas
IV Sympathetic Nervous System Tumors
III CNS and Miscellaneous Intracranial and Intraspinal Neoplasm
II Lymphomas and Reticuloendothelial Neoplasm
I Leukaemia
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Warning Signs of Cancer in Children
• Continued, unexplained weight loss
• Headaches with vomiting in the morning
• Increased swelling or persistent pain in bones or joints
• Lump or mass in abdomen, neck, or elsewhere
• Development of whitish appearance in pupil
• Recurrent fevers not caused by infections
• Excessive bruising or bleeding
• Noticeable paleness or prolonged tiredness
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Çocuk kanserlerinde başlıca belirti ve bulgular
• Solukluk, halsizlik• Sık ateşlenme• Kilo kaybı• Gelişme geriliği• Kanama bulguları (peteşi, ekimoz)• Burun, dişeti kanamaları
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Çocuk kanserlerinde başlıca belirti ve bulgular
• Başağrısı, kusma• Ateşsiz havale geçirme• Dengesizlik, yürüme bozukluğu• Gözde kayma, görme bozukluğu• Göz bebeğinde parlaklık
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ÇOCUKLUK ÇAĞI TÜMÖRLERİNDE UYARICI BULGU VE BELİRTİLER
• Kemik, eklem ağrıları• Enfeksiyon tedavisine rağmen sebat eden
öksürük, nefes darlığı• İdrarda kan gelmesi• İdrar ve dışkılamada zorlanma
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Çocuk kanserlerinde başlıca belirti ve bulgular
• Yorgunluk• Solukluk• Ateş• Kanama bulguları• Ağırlık kaybı• Geçmeyen öksürük• Tedaviye rağmen geçmeyen
enfeksiyon bulguları
• Lenfadenomegali• Kafa içi kitleler• Mediasten kitleleri• Abdominal kitleler• Urogenital kitleler• Ekstremite kitleleri• Endokrin belirtiler• Diğer belirtiler
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TANI
• Muayene• Kan tetkikleri (TKS, förmül, biyo.,tm belirteçleri)• Kemik iliği tetkiki (bazı kanserlerde)• Görüntüleme yöntemleri• Patolojik tanı için örnek alınması
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PEDIATRIC ÇAĞI BRAIN TUMORSPEDIATRIC ÇAĞI BRAIN TUMORS• Mostly• embriyonal • infratentorial (≤2 yr supratentorial)• CNS seeding• Treatment
• Surgery Radiotherapy Chemotherapy
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-Supratentorial Astrositomlar (yüksek grad %10,düşük grad %20)SPNETEpendimom-InfratentorialMedulloblastomBeyin sapı gliomuEpandimoma Serebellar astrositoma-MidlineOptik kiazma gliomları Kraniofarinjioma Pineal bölge tümörleri
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SUPRATENTORİAL HEMİSFERİK
• Konvulziyon • Hemiparezi• Bir yanda duyu kusuru• Hiperrefleksi• Frontal lob-kişilik ve motor fonksiyonlar• Parietal lob-okuma, kontralateral ekstremiteler• Temporal lob-afazi, parsiyel kompleks konv.• Oksipital lob-görme alanı bozukluğu
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İNFRATENTORİAL(arka çukur, beyin sapı, serebellar hemisfer)
• Ataksi• Dismetri, disdiadokinezi• Beyin sapı-kranial sinir tutulumları
hemiparezi• Serebellar hemisferlerde-lateralizasyon bulgusu
(appendiküler dismetri)
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Medulloblastoma
Evreleme Kranium MRSpinal aks MR kontrastlıBOS sitoloji
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Brain stem glioma
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LOKALİZE BULGU VE BELİRTİLER
SUPRATENTORİAL ORTA HAT
• Optik kiazma tm-görme alanı defekti• Hipotalamus (SÇ) diensefalik sendrom • Kraniofarenjioma-endokrin sorunlar
görme alanı boz hidrosefali
• Pineal bölge tm- Parinaud sendromuyukarı bakış kısıtlıdilate pupillerkonverjans nistagmusukapak retraksiyonu
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OPTİC GLİOMAKebudi R,Upadhyaya M,Tuncer S,et al. A novel mutation in the NF-1 gene in two siblings with neurofibromatosis type 1 and bilateral optic pathway glioma. Pediatric Blood and Cancer 2008
NF EUROPE 2012İstanbul
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GCT GLİOM
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80
60
40
20
0Astrocytoma PNET Other gliomas
70
74
5550
47
57
39
56
SEER Data - 5-year relative survival rate by histology and time period
Ependymoma
1975-85
1985-95
All CNS
6065
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PEDIATRIC LYMPHOMASPEDIATRIC LYMPHOMAS%60 Non-Hodgkin , % 40 Hodgkin%60 Non-Hodgkin , % 40 HodgkinHodgkin Lymphoma Reed Sternberg hücresi
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Non-Hodgkin lymphomas- fastest doubling time (Burkitt)- tümör lysis syndrome!
50%
33%
17%
Küçük çentiksizhücreli (burkitt)
Lenfoblastik
Büyük hücreli
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Non-Hodgkin : nonlenfoblastik B cell Burkitt/Burkitt
Treatment: Chemotherapy
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PET-CT
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Neuroblastoma
Treatment•Surgery•Chemot. + Radiotherapy•Biologic tr. Retinoik asid Fenretidine GD + Il-2
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Nöroblastoma SonuçlarımızKebudi R, et al. Istanbul Pediatric Oncology Group Neuroblastoma results. 40th Congress of the International Society of Pediatric Oncology, Berlin, 2008.
• 173 hasta, median yaş 26 ay (1-186).
Primer Yerleşim • abdomen 125 (surrenal 110)
(73%) • mediastinum 22 (12.2%)• paraspinal 18 (10%) • Diğer 8 (4.8%)
• Evre: (INSS)• Evre I 16 (9%) • Evre II 20 (12%)• Evre III 32 (19%) • Evre IV 101 (58 %) • Evre IVS 4 (2%).
SAĞKALIM5 ve 10 yıl S
Tüm grup %73 ve 54
10 yıl survivalEvre I,II % 100Evre III % 54Evre IV % 26 (p= 0.0001 ).Yaş <12 ay
< 18 ay
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-Wilms tmTreatmentSurgeryradikal nefrektomi
Chemot.Radioth.(stage3,4;stage 1,2 poor histology)
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LIVER TM
• Hepatoblastoma• HCC• FNH• Hamartom• İnf. Myofibroblastik tm
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Pre-operative chemotherapy
2 - 3 months
Post-operative chemotherapy
DELAYED SURGERY
B
I
O
P
S
Y
2 months
SIOPEL TREATMENT STRATEGY
Hepatoblastoma - SIOPEL Experience
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0.0
0.2
0.4
0.6
0.8
1.0
6 12 18 24 30 36 42 48
SIOPEL 2 pilot study -Treatment results - 5-year survival data by risk category
monthsOverall survival
Progression free survival
%
Standard risk
High risk
CDDP alone
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Intraabdominal malignancies• Nöroblastom• Wilms• Lenfoma• Rabdomyosarkom• Ekstraskeletal Ewing• GCT vd.• EVALUATION• Ultrason• Direkt grafiler• BT / MRI• Hemogram• Kemik iliği• Biyokimya • Tümör belirteçleri:NSE,VMA,
AFP,b-HCG• İğne/trucut biyopsileri• Laparaskopi/Laparatomi
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SOFT TISSUE SARCOMASSOFT TISSUE SARCOMAS
All agesAll ages % 0.7% 0.7 2/1052/105< 15 yr: All malignancies < 15 yr: All malignancies % 7% 7 8.8/1068.8/106
NonRMS NonRMS % 3 % 3Rabdomiyosarkom Rabdomiyosarkom Median age 5yr--(2-6) ve (15-19) NONRabdomiyosarkomaNONRabdomiyosarkoma
Ewing sarcoma family tmEwing sarcoma family tm
OtherOther
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RABDOMIOSARCOMA1. >% 50 Rabdomiyosarkom
2.Survival % 25( 1970) % 70 (2000)Tedavi:S ± RT + Chemo3. Prognostic factors
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Rabdomiyosarkom - YerleşimHead and neck Paramengeal
Orbital NPNOBB
Genitourinary ParatestikülerVaginal, uterus
Mesane/prostat (MP)ExtremitiesOther
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İ.Ü.Onkoloji Enstitüsü, Pediatrik Onkoloji B.D.1989 –2002 Demografik veriler
N=95, E:K 1:1, Median yaş 5 (1-18) yaş7 yıllık Sağkalım % 61 Evre Sıklık Sağkalım(7 y)Histoloji Sıklık S (7y)Evre 1 % 29 %100 Alveolar /ind % 37 %49Evre 2 % 15 %66 Embryonal %46.5 %70Evre 3 % 48 %62 Botrioid %16.5 %46Evre 4 % 8 %0Yerleşim orbita,NPBB % 100, Paratestiküler%90, vagen-uterus % 80, Mesane-prostat % 51,
paramenengeal %42,ekstremite %27Kebudi R,et al.Evaluation of children with rhabdomyosarcoma.Proceedings of American Society of Clinical Oncology 22: 802,
2003
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RABDOMİYOSARKOM
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OSTEOSARCOMA• Most 10-20 yr• metaphysis• distal femur, proximal tibia ve proximal humerusta• % 20 metastaz at diagnosis (lungs, bone)• Treatment
Neoadjuvan chemo+S + Adjuvan chemo
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OSTEOSARCOMA
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Ewing Sarcoma
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Ekstraskeletal Ewing Sarcoma
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Retinoblastoma
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Langerhans Cell Histiocytosis
Kebudi,R., Ayan,İ., Görgün,Ö, Özger,H.,Darendeliler E: Evaluation of 36 children with Langerhans cell histiocytosis with bone involvement. Medical and
Pediatric Oncology 41: 338, 2003
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Tuncer S, Kebudi R, Peksayar G, et al. Congenital Mesenchymal chondrosarcoma of the orbit: Ophtalmology 111:1016-22, 2004
AMLLCH
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Mediastinal massesAnteriorTimus TiroidTeratom (Germ hücreli tümörler) Lenfoma (Hodgkin,NHL) T cell leukemiaMiddle(%30)Lenfomalar (Hodgkin,NHL)Posterior Mediastinum (%40)Nöroblastom, Nörofibrom/Schwannom
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TM MARKERS
• GERM CELL TM• AFP- Endodermal sinus tm• B-HCG-Koriokarsinom• AFP + BHCG- embriyonal karsinom
• Neuroblastoma-serum NSE, urine VMA
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Genitoüriner / perianal masses
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Nazofarenks Ca
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Late effects
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Pediatric cancer
• Survival % 65-70
EARLY DIAGNOSISMULTIDISIPLINARY APPROACHCANCER CENTER
TEAM WORK