2 CANCER IN CHILDHOODUS figures show total of 146 cancers per million children aged 0-14 per yearALLBRAIN TUMOURSNEUROBLASTOMANHLWILMS TUMOURAMLHODGKIN’S DISEASERHABDOMYOSARCOMARETINOBLASTOMAOSTEOSARCOMAEWING’S TUMOUROTHERS
3 CHILDHOOD CANCER AT RCCH Approximately 130 new cases per yearMany present with advanced diseaseMany have presented to the health service several times over the preceding weeks or months !These delays make treatment more difficult … and adversely effects prognosis.
4 SPECTRUM AT RCCHNumbers represent actual number of tumours for 2003 / 2004 / 2005ALLBRAIN TUMOURSNHLAMLWILMS TUMOURNEUROBLASTOMARHABDOMYOSARCOMAGERM CELL TUMOURHODGKIN’S DISEASEHEPATOBLASTOMABONE TUMOURSRETINOBLASTOMAOTHER
5 ONCOLOGY FOR THE PAEDIATRICAN: What’s the central message ? EARLY DIAGNOSISMEANS …Improved prognosisLess treatment related morbidityNEEDS …Working knowledge of common tumoursHigh index of suspicion
6 IMPROVED PROGNOSIS “WHAT A DIFFERENCE A DAY (OR TWO) MAKES” FAVOURABLE HISTOLOGYWILMS’ TUMOUR
9 LESS TOXICITY “SMALLER OMELETTE … LESS EGGS” WILMS’ TUMOUR TREATMENT…Surgery (primary or delayed) for allChemotherapyStage I or II: Vincristine + DactinomycinStage III or IV: Add DOXORUBICINRENAL BED RADIOTHERAPY for local Stage IIIPULMONARY RADIOTHERAPY for lung metastases
11 NEPHROBLASTOMA Named for MAX WILMS Derived from the metanephros = hence the classic triphasic nature = epithelial / blastemal / stromalAssociationsOvergrowth = BWS / HemihypertrophyNon-overgrowth = Aniridia / WAGR / Denys-DrashAge 0-5yrs (median 3yrs)Presents with Mass / Pain / Haematuria / ParaneoplasiasRenal mass … L>R (not across midline) 10% bilateralMetastasis … lungs / liver / brain / (bone)
12 STAGING WILMS’ TUMOUR Stage I ... confined to kidney Stage II ... through capsuleStage III ... residual tumourLocal spreadNodesIVC ThrombusStage IV ... metastatic diseaseStage V bilateral disease
13 DIAGNOSIS AND MANAGEMENT DIAGNOSIS and STAGINGFBC / Urea & Creatinine / LDHUrineUltrasound and CT AbdomenCXR and CT ChestBiopsy if deemed inoperable or protocol calls for pre-op chemoMANAGEMENTTwo schools (NWTS and SIOP)Surgery + Chemo +/- XRTSame results ! In fact …
14 So good that we’re deintensifying treatment !! FAVOURABLE HISTOLOGYWILMS’ TUMOUR
15 NEUROBLASTOMADerived from primitive neural crest cells of the sympathetic nervous systemHence they are found along the sympathetic chain1/3 adrenal 1/3 abdominopelvic 1/3 thoracic or cervicalAge 0-3yrs [median of 17m] [40% are infants]Prognosis inversely related to age … Younger kids usually have lower grade tumours (some of which regress spontaneously) in more favourable sites (cervicothoracic) and are much less likely to metastasizeStaging:I: confined to organII: extends outside organ (+/- nodes) NOT across midlineIII: across midline … involving contralateral nodesIV: Metastasis to Liver / Bone and BM / SkinIVS: I/II with remote disease in liver, skin, bone marrow (<10%)
16 CLINICAL PRESENTATION Abdominal mass > 50% … usually smooth and hard (crosses midline)Pelvic MassMediastinal or cervical massMass effect … proptosis / superior mediastinal syndrome / UAO /spinal cord compressionBone marrow … anaemia / thrombocytopaenia / bone pain with limpMetabolic (catecholamines and vasoactive intestinal peptides) … sweating / diarrhoea & vomiting / hypertensionBluish skin marks (typically blanch)Opsoclonus-myoclonus / Cerebellar ataxia
17 DIAGNOSIS AND MANAGEMENT DIAGNOSIS and STAGINGFBC / Urea & Creatinine / LDHUrinary catecholamines (HVAs and VMAs)Calcified mass on plain AXRImaging of abdomen, chest and possibly spineBone Marrow / MIBG / Bone ScanBiopsy can be avoided if BM involved !MANAGEMENTSurgery +/- Chemotherapy
18 RHABDOMYOSARCOMA Commonest soft tissue sarcoma of childhood Derived from primitive muscleAge 2-15yrsPrognosis varies withHistology: Embryonal > AlveolarSite: Orbit > Paratesticular > Vaginal > Bladder / Prostate > Retroperitoneal > Extremity > ParameningealStageStaging:I to IV based on complex system using TNM and SiteIRS Grouping now generally followed …Group I completely excised Group II microscopic residuaGroup III macroscopic residua Group IV metastases
19 DIAGNOSIS AND MANAGEMENT DIAGNOSIS and STAGINGFBC / Urea & Creatinine / LDHLocal imaging …CT for abdomen / MRI for parameningeal tumoursCXR and CT chestBone MarrowMANAGEMENTChemotherapy with Surgery +/- XRT
20 HEPATOBLASTOMA DIAGNOSIS MANAGEMENT Commonest paediatric liver tumour Age usually under 18 monthsPresents as hepatomegaly with elevated FPMetastasizes locally and to lungsDIAGNOSISFBC / Renal and liver functions / FPCT abdmen and chestMANAGEMENTChemotherapySurgery
21 CLINICAL SCENARIOS IN SOLID PAEDIATRIC TUMOURS AN APPROACH FOR THE GENERAL PAEDIATRICIAN
22 1. PANCYTOPAENIA LEUKAEMIA LYMPHOMA or NEUROBLASTOMA ASK ABOUT … bone painWATCH OUT FOR …swollen gumslymph nodesabdominal massesproptosis
23 1. PANCYTOPAENIA MUST exclude HIV infection … LEUKAEMIA (or LYMPHOMA) FBC: Hb Plt WCC / … look for blastsChemistry: LDH (with a normal ALT) and Urate CXR: mediastinal mass in T-cell lymphomaNEUROBLASTOMALook for an Abdominal Mass !FBC: typically anaemic with preserved plateletsChemistry: LDH (with a normal ALT) and urinary HVAsXrays: lytic bone lesions or calcified abdominal mass
24 1. PANCYTOPAENIA WHO SHOULD DO THE BONE MARROW ? Generally the receiving hospital but this matter should always be discussed!LEUKAEMIA (or ANY SUSPECTED MALIGNANCY)Aspirates and trephines Flow cytometry Cytogenetics Ig gene rearrangements by PCR FISH for translocationsComplicated set of investigations: leave for referral centre !OTHER INDICATIONSDisseminated TB / ITP / Storage disorders.Probably fine to do.
25 2. BONE PAIN NOT localised Wakes the child Limp or Reluctance to bear weightA toddler who stops walkingBackache!!
26 2. BONE PAIN LEUKAEMIA or LYMPHOMA Again, malignancies involving the bone marrow…LEUKAEMIA or LYMPHOMANEUROBLASTOMARemember … if local pain and swelling (especially around the knee joint)…BONE TUMOURS eg. OSTEOGENIC SARCOMA
27 3. ABDOMINAL MASS from 6m to 6y … ABDOMINAL MASS is a tumour until proven otherwiseALWAYS CONSIDER SEDATING THE PATIENT IF YOU CAN”T PALPATE THE ABDOMEN !
28 3. ABDOMINAL MASS REFER WILMS TUMOUR HEPATOBLASTOMA NEUROBLASTOMA flank mass / typical imaging / LDHHEPATOBLASTOMAhepatomegaly / typical imaging / Feto-proteinNEUROBLASTOMAadrenal mass / typical imaging / LDH and HVAs? signs of metastatic involvementBURKITT LYMPHOMAcentral abdominal mass / nodes on imaging / LDHNo need for BIOPSY !!REFER
29 (3. PELVIC MASS) REFER GERM CELL TUMOUR BURKITT LYMPHOMA Feto-proteinBURKITT LYMPHOMA LDH and urateRHABDOMYOSARCOMA LDHNEUROBLASTOMANo need for BIOPSY !!REFER
30 4. AN UNEXPLAINED MASSSITESHead and NeckLimbsTestis
31 5. UNEXPLAINED NEUROLOGY Headache > 2 weeksEarly morning vomitingAtaxia / unsteady gaitCranial Nerve PalsyBRAIN TUMOURS (watch out for neurocutaneous syndromes)Posterior fossa … ataxia and cranial nerve palsiesSupratentorial … headache and vomiting hemiplegiaRHABDOMYOSARCOMA (often occult in the sinuses)Lower cranial nerve palsies eg. VI, VII, IX, XIINEUROBLASTOMA (secondary to skull infiltration)Commonly VI nerve palsy
32 7. EYE CHANGES RETINOBLASTOMA White ReflexRecent Onset SquintLoss of VisionRETINOBLASTOMAProptosisLEUKAEMIA especially Acute Myeloid LeukaemiaNEUROBLASTOMARHABDOMYOSARCOMA
33 7. EYE CHANGES Leukocoria REFER to an Ophthalmologist urgently for slit lamp examination
34 7. EYE CHANGESProptosisCONSIDER Leukaemia / Neuroblastoma / Rhabdomyosarcoma … abnormal FBC means diagnosis might be made on BM examination … REFEROTHERWISE REFER to an Ophthalmologist for urgent biopsy
35 IN SUMMARY … REMEMBER THE WARNING SIGNS Pallor plus BleedingPersistent Fever / Apathy / Weight LossBone PainAdenopathyUnexplained Neurological SignsUnexplained MassEye Changes
36 THE BOTTOM LINE Be aware of the clinical spectrum of childhood cancer Don’t waste time with special investigations … Seek expert adviceRefer cases early … and URGENTLY !