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’ TUMOUR TREATMENT…
Surgery (primary or delayed) for all
Chemotherapy
Stage I or II: Vincristine + Dactinomycin
Stage III or IV: Add DOXORUBICIN
RENAL BED RADIOTHERAPY for local Stage III
PULMONARY RADIOTHERAPY for 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 WILMS’ TUMOUR 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

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 DIAGNOSIS MANAGEMENT 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 LEUKAEMIA or LYMPHOMA
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 REFER WILMS TUMOUR HEPATOBLASTOMA NEUROBLASTOMA
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) REFER GERM CELL TUMOUR BURKITT LYMPHOMA
 Feto-protein
BURKITT LYMPHOMA
 LDH and urate
RHABDOMYOSARCOMA
 LDH
NEUROBLASTOMA
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 RETINOBLASTOMA
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 THE WARNING 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 !