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PAEDIATRIC SOLID TUMOURS

PAEDIATRIC SOLID TUMOURS. Alan Davidson Haematology - Oncology Red Cross Children’s Hospital. CANCER IN CHILDHOOD. US figures show total of 146 cancers per million children aged 0-14 per year. ALL BRAIN TUMOURS NEUROBLASTOMA NHL

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PAEDIATRIC SOLID TUMOURS

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  1. PAEDIATRIC SOLID TUMOURS Alan Davidson Haematology - Oncology Red Cross Children’s Hospital

  2. CANCER IN CHILDHOOD • US figures show total of 146 cancers per million children aged 0-14 per year ALL BRAIN TUMOURS NEUROBLASTOMA NHL WILMS TUMOUR AML HODGKIN’S DISEASE RHABDOMYOSARCOMA RETINOBLASTOMA OSTEOSARCOMA EWING’S TUMOUR OTHERS

  3. CHILDHOOD CANCER AT RCCH • Approximately 130 new cases per year • Many present with advanced disease • Many 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 RCCH Numbers represent actual number of tumours for 2003 / 2004 / 2005 ALL BRAIN TUMOURS NHL AML WILMS TUMOUR NEUROBLASTOMA RHABDOMYOSARCOMA GERM CELL TUMOUR HODGKIN’S DISEASE HEPATOBLASTOMA BONE TUMOURS RETINOBLASTOMA OTHER

  5. ONCOLOGY FOR THE PAEDIATRICAN: What’s the central message ? • EARLY DIAGNOSIS • MEANS … • Improved prognosis • Less treatment related morbidity • NEEDS … • Working knowledge of common tumours • High index of suspicion

  6. IMPROVED PROGNOSIS“WHAT A DIFFERENCE A DAY (OR TWO) MAKES” FAVOURABLE HISTOLOGY WILMS’ TUMOUR

  7. BURKITT’S LYMPHOMA

  8. EWING’S SARCOMA

  9. LESS TOXICITY“SMALLER OMELETTE … LESS EGGS” WILMS’ TUMOURTREATMENT… • Surgery (primary or delayed) for all • Chemotherapy • Stage I or II: Vincristine + Dactinomycin • Stage III or IV: AddDOXORUBICIN • RENAL BED RADIOTHERAPY for local Stage III • PULMONARY RADIOTHERAPYfor lung metastases

  10. CLASSIFYING CHILDHOOD MALIGNANCY • LEUKAEMIAS • ALL / AML / CML • EMBRYONAL TUMOURS • Neuroblastoma • Nephroblastoma • Rhabdomyosarcoma • Hepatoblastoma • Retinoblastoma • PNET • Germ Cell Tumour • BRAIN TUMOURS • Seperate textbook ! • BONE TUMOURS • Osteogenic Sarcoma • Ewing’s Sarcoma • RARE TUMOURS • Thyroid Ca / Melanoma

  11. NEPHROBLASTOMA • Named for MAX WILMS • Derived from the metanephros = hence the classic triphasic nature = epithelial / blastemal / stromal • Associations • Overgrowth = BWS / Hemihypertrophy • Non-overgrowth = Aniridia / WAGR / Denys-Drash • Age 0-5yrs (median 3yrs) • Presents with Mass / Pain / Haematuria / Paraneoplasias • Renal mass … L>R (not across midline) 10% bilateral • Metastasis … lungs / liver / brain / (bone)

  12. STAGING • Stage I ... confined to kidney • Stage II ... through capsule • Stage III ... residual tumour • Local spread • Nodes • IVC Thrombus • Stage IV ... metastatic disease • Stage V ... bilateral disease WILMS’ TUMOUR

  13. DIAGNOSIS AND MANAGEMENT • DIAGNOSIS and STAGING • FBC / Urea & Creatinine / LDH • Urine • Ultrasound and CT Abdomen • CXR and CT Chest • Biopsy if deemed inoperable or protocol calls for pre-op chemo • MANAGEMENT • Two schools (NWTS and SIOP) • Surgery + Chemo +/- XRT • Same results ! In fact …

  14. So good that we’re deintensifying treatment !! FAVOURABLE HISTOLOGY WILMS’ TUMOUR

  15. NEUROBLASTOMA • Derived from primitive neural crest cells of the sympathetic nervous system • Hence they are found along the sympathetic chain • 1/3 adrenal 1/3 abdominopelvic 1/3 thoracic or cervical • Age 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 metastasize • Staging: • I: confined to organ • II: extends outside organ (+/- nodes) NOT across midline • III: across midline … involving contralateral nodes • IV: Metastasis to Liver / Bone and BM / Skin • IVS: I/II with remote disease in liver, skin, bone marrow (<10%)

  16. CLINICAL PRESENTATION • Abdominal mass > 50% … usually smooth and hard (crosses midline) • Pelvic Mass • Mediastinal or cervical mass • Mass effect …proptosis / superior mediastinal syndrome/ UAO / spinal cord compression • Bone marrow … anaemia / thrombocytopaenia /bone pain with limp • Metabolic(catecholamines and vasoactive intestinal peptides) … sweating / diarrhoea & vomiting / hypertension • Bluish skin marks (typically blanch) • Opsoclonus-myoclonus / Cerebellar ataxia

  17. DIAGNOSIS AND MANAGEMENT • DIAGNOSIS and STAGING • FBC / Urea & Creatinine / LDH • Urinary catecholamines (HVAs and VMAs) • Calcified mass on plain AXR • Imaging of abdomen, chest and possibly spine • Bone Marrow / MIBG / Bone Scan • Biopsy can be avoided if BM involved ! • MANAGEMENT • Surgery +/- Chemotherapy

  18. RHABDOMYOSARCOMA • Commonest soft tissue sarcoma of childhood • Derived from primitive muscle • Age 2-15yrs • Prognosis varies with • Histology: Embryonal > Alveolar • Site: Orbit > Paratesticular > Vaginal > Bladder / Prostate > Retroperitoneal > Extremity > Parameningeal • Stage • Staging: • I to IV based on complex system using TNM and Site • IRS Grouping now generally followed … Group I completely excised Group II microscopic residua Group III macroscopic residua Group IV metastases

  19. DIAGNOSIS AND MANAGEMENT • DIAGNOSIS and STAGING • FBC / Urea & Creatinine / LDH • Local imaging … CT for abdomen / MRI for parameningeal tumours • CXR and CT chest • Bone Marrow • MANAGEMENT • Chemotherapy with Surgery +/- XRT

  20. HEPATOBLASTOMA • Commonest paediatric liver tumour • Age usually under 18 months • Presents as hepatomegaly with elevated FP • Metastasizes locally and to lungs • DIAGNOSIS • FBC / Renal and liver functions / FP • CT abdmen and chest • MANAGEMENT • Chemotherapy • Surgery

  21. CLINICAL SCENARIOS IN SOLID PAEDIATRIC TUMOURS AN APPROACH FOR THE GENERAL PAEDIATRICIAN

  22. 1. PANCYTOPAENIA • LEUKAEMIA • LYMPHOMA or NEUROBLASTOMA • ASK ABOUT … • bone pain • WATCH OUT FOR … • swollen gums • lymph nodes • abdominal masses • proptosis

  23. 1. PANCYTOPAENIA • MUST exclude HIV infection … • LEUKAEMIA (or LYMPHOMA) • FBC: Hb Plt  WCC / … look for blasts • Chemistry: LDH  (with a normal ALT) and Urate  • CXR: mediastinal mass in T-cell lymphoma • NEUROBLASTOMA • Look for an Abdominal Mass ! • FBC: typically anaemic with preserved platelets • Chemistry: LDH  (with a normal ALT) and urinary HVAs • Xrays: 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 translocations • Complicated set of investigations: leave for referral centre ! • OTHER INDICATIONS • Disseminated TB / ITP / Storage disorders. • Probably fine to do.

  25. 2. BONE PAIN • NOT localised • Wakes the child • Limp or Reluctance to bear weight • A toddler who stops walking • Backache!!

  26. 2. BONE PAIN • Again, malignancies involving the bone marrow… • LEUKAEMIA or LYMPHOMA • NEUROBLASTOMA • Remember … 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 otherwise ALWAYS CONSIDER SEDATING THE PATIENT IF YOU CAN”T PALPATE THE ABDOMEN !

  28. 3. ABDOMINAL MASS • WILMS TUMOUR • flank mass / typical imaging /  LDH • HEPATOBLASTOMA • hepatomegaly / typical imaging / Feto-protein • NEUROBLASTOMA • adrenal mass / typical imaging /  LDH and HVAs • ? signs of metastatic involvement • BURKITT LYMPHOMA • central abdominal mass / nodes on imaging /  LDH No need for BIOPSY !! REFER

  29. (3. PELVIC MASS) • GERM CELL TUMOUR  Feto-protein • BURKITT LYMPHOMA  LDH and urate • RHABDOMYOSARCOMA  LDH • NEUROBLASTOMA  LDH No need for BIOPSY !! REFER

  30. 4. AN UNEXPLAINED MASS • SITES • Head and Neck • Limbs • Testis

  31. 5. UNEXPLAINED NEUROLOGY • Headache > 2 weeks • Early morning vomiting • Ataxia / unsteady gait • Cranial Nerve Palsy • BRAIN TUMOURS (watch out for neurocutaneous syndromes) • Posterior fossa … ataxia and cranial nerve palsies • Supratentorial … headache and vomiting  hemiplegia • RHABDOMYOSARCOMA (often occult in the sinuses) • Lower cranial nerve palsies eg. VI, VII, IX, XII • NEUROBLASTOMA (secondary to skull infiltration) • Commonly VI nerve palsy

  32. 7. EYE CHANGES • White Reflex • Recent Onset Squint • Loss of Vision • RETINOBLASTOMA • Proptosis • LEUKAEMIA especially Acute Myeloid Leukaemia • NEUROBLASTOMA • RHABDOMYOSARCOMA

  33. 7. EYE CHANGES • Leukocoria • REFER to an Ophthalmologist urgently for slit lamp examination

  34. 7. EYE CHANGES • Proptosis • CONSIDER Leukaemia / Neuroblastoma / Rhabdomyosarcoma … abnormal FBC means diagnosis might be made on BM examination … REFER • OTHERWISE REFER to an Ophthalmologist for urgent biopsy

  35. IN SUMMARY … REMEMBER THEWARNING SIGNS • Pallor plus Bleeding • Persistent Fever / Apathy / Weight Loss • Bone Pain • Adenopathy • Unexplained Neurological Signs • Unexplained Mass • Eye Changes

  36. THE BOTTOM LINE • Be aware of the clinical spectrum of childhood cancer • Don’t waste time with special investigations … Seek expert advice • Refer cases early … and URGENTLY !

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