Leukaemia 35% Lymphoma 14%

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1 Distribution ib ti of Cancers in Children under 15 years Leukaemia 35% Lymphoma 14% Neuroblastoma 9% Other 5% Liver 1% Retinoblastoma 3% Bone and STS 15% CNS 20% Wilms' 8% 30-40% Mortality

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3 Germ Cell Tumours

4 Germ Cell Tumours WHO Classification Morphological and immunophenotypic homologues of gonadal and other extra- neuroaxial germ cell tumours

5 Germ cell tumours Teratoma Mature Immature With malignant GCT components Germinoma Embryonal carcinoma Endodermal sinus (yolk sac) tumour Choriocarcinoma Gonadoblastoma

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7 Germ cell tumours Extragonadal tumours Sacrococcygeal Mediastinal Abdominal Intracranial Head and neck Vaginal Gonadal tumours Ovarian Testicular

8 Germ Cell Tumours CNS Pineal, Suprasellar, Basal Ganglia Can be multifocal Are midline; brain, mediastinum, sacrum, (gonads) when extra-gonadal, testis and ovaries Infants:sacrococcygeal

9 Germ Cell Tumour Germinoma Non germinomatous Germ Cell Tumour Teratoma Immature Teratoma Embyronal Carcinoma Yolk Sac Tumour Choriocarcinoma Mixed Germ Cell Tumour

10 Germ Cell Tumours Increased in Klinefelters Syndrome

11 Tumour Markers AFP bhcg

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14 Germ cell tumours - sacrococcygeal Sacrococcygeal Make up 40% of GCT Infants 50%, and 70% are females Constipation, neurologic abnormalities of bladder or lower extremities Presacral mass Infancy 65% benign, 5% mature, 30% malignant

15 Germ cell tumours - sacrococcygeal Type I mainly external with minimal presacral component Type II external with significant internal presacral component Type III external, predominant mass internal with extension into abdomen Type IV entirely presacral, without external presentation

16 Germ cell tumours - sacrococcygeal Type I - most common and usually not malignant Type IV 10%, and malignant. Usually presents with constipation. Sometimes loss of natal cleft, bruising. If a mass is felt anteriorly, most likely RMS. If posteriorly, DDx GCT, NBL, chordoma

17 Germ cell tumours gonadal (ovary) Ovarian GCT s account for 60-90% of all ovarian tumours Benign teratomas 65% Embryonal (immature) teratomas 5% Malignant 30% Others are epithelial cancers /fibrosarcomas/ granulosa-thecal/ undifferentiated

18 Germ cell tumours gonadal (ovary) Of malignant tumours, 30% endodermal sinus tumours rest are often mixtures of dysgerminomas, choriocarcinomas, embryonal carcinomas etc Presentation Abdo pain, usually chronic Nausea/vomiting/+fever ~ torsion Always think of ovarian mass in young girls/women if also thinking of appendicitis. Calcification/mass. βhcg production ~ early menarche/mimics pregnancy

19 Germ cell tumours gonadal (ovary) Elevated hcg levels seen in embryonal CA and choriocarcinoma AFP raised in endodermal sinus tumour DDx Ovarian cysts Appendicitis Tubal pregnancy Pelvic sarcoma/lymphoma/non-gct ovary Pelvic abscess

20 Germ cell tumours gonadal (testis) Testis 70% are GCT, 30% non-germ cell Paratesticular RMS/ALL/lymphoma/stroma sex cord tumour (ie Sertoli, leydig, juvenile granuloas cell) Of the GCT ~ Make up 1% of all childhood cancer Most common teratoma Most common malignancy endodermal sinus tumour (yolk sac tumour). Seminomas rare < 5%

21 Germ cell tumours gonadal (testis) Clinical ~ Slowly increasing painless mass 25% have associated hydrocele so dx sometimes tricky? Undescended testis : increased incidence of malignancy in the contralateral undescended testis in patients with unilateral cryptorchidism

22 Staging and Treatment Serum AFP and βhcg Local imaging especially for nodes. Look for calcification. Doesn t mean benign but points to diagnosis eg CXR, mediastinal mass, Ca = GCT Abdominal scan liver/retroperitoneum CXR/chest CT/bone scan

23 Staging Local Lungs Bones Peritoneal deposits Spine/CSF in brain Beware seeding from VP shunts

24 Treatment Surgery Chemotherapy Platinum based CNS:Radiation (CSI/Whole ventricular and local)

25 Prognosis Non CNS Testis / ST I Surg Only EFS : Testis / ST II Standard d PEB EFS: 100 Ovary / ST I Standard PEB EFS : Ovary / ST II Standard PEB EFS: Sacrococcygeal Surgery EFS 95%

26 Hepatoblastoma

27 Most common malignant hepatic tumour of childhood

28 Rare >5y 75% <1y Unknown Possible factors Epidemiology Etiology BW<1000g Fetal alcohol syndrome OCP use during pregnancy Genetic abnormalities Beckwith-Wiedemann Isolated hemihypertrophy h Familial polyposis IGF2 deregulation 11p15 APC gene chr 5

29 Pathology Pure epithelial - fetal Pure epithelial embryonal Mixed epithelial/mesenchymal excellent prog intermediate prog Usually localised R lobe Mets lung porta hepatis

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32 Clinical Abdominal mass Pain Constitutional symptoms Investigations Alpha feto protein >90% cases Hb Plt CXR AXR CT MRI

33 Staging Extent of local disease pretext, resectability, rupture Metastatic disease : lungs

34 PRETEXT/POSTTEXT Group Number of Affected Liver Sections 1 Tumor involves only 1 liver section; 3 adjoining sections are free of tumor. 2 Tumor involves 2 adjoining liver sections; 2 adjoining sections are free of tumor. 3 Tumor involves 3 adjoining sections or 2 nonadjoining sections; 1 section or 2 nonadjoining sections are free of tumor. 4 Tumor involves all 4 liver sections; there is no section free of tumor. Any PRETEXT/POST-TEXT Group can be annotated with the following: +V Involvement of vena cava or all 3 hepatic veins +P Involvement of main portal vein bifurcation or both right and left portal veins +C Involvement of caudate lobe +E Extrahepatic contiguous tumor +M Distant metastatic disease

35 Management Surgery Adjuvant chemotherapy Cisplatin Doxorubicin Cisplatin 5FU VCR RT no role Liver transplant if unresectable

36 Prognosis Stage 1 and 2 90%+ Stage 3 60% Stage % Prognostic factors Resectability of gross disease diffuse vs localised primary Presence of mets Histology epithelial differentiation AFP >2logs with first course chemo