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PAEDIATRIC SOLID TUMOURS
Alan DavidsonHaematology - Oncology
Red Cross Children’s Hospital
23.22.13.74.655.37.18.68.7
11.332.4
34.6
• 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
CANCER IN CHILDHOOD
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.
1 6
0
5
4
3
5
4
5
9
8
1 1
1 6
1 7
1 6
4
2
1
6
3
6
5
5
1 6
1 3
1 4
1 7
1 0
3
1
4
3
7
6
7
7
8
1 2
1 5
1 5
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
SPECTRUM AT RCCH
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
IMPROVED PROGNOSIS“WHAT A DIFFERENCE A DAY (OR TWO) MAKES”
0 50 100 150 200 250 300 350
T ime - months
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cum
ulative Proportion S
urviving
S tage I 94.4% S tage II 96.2% S tage III 84.9% S tage IV 54.2%
Comple te CensoredCh i-squa re = 22 .898 d f = 3 p = 0 .00004
FAVOURABLE HISTOLOGY WILMS’ TUMOUR
O ver all 5 -year S ur vi valLM B Chemother apy
1988-2004[Log R ank p value 0 .0 6 ]
Complete Cens ored
Stage II and III [88.2% ]
Stage IV [66.0% ]
0 20 40 60 80 100 120 140 160 180 200 220
Time - months
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cum
ulative Proportion S
urviving
BURKITT’S LYMPHOMA
EWING’S SARCOMA
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
LESS TOXICITY“SMALLER OMELETTE … LESS EGGS”
• LEUKAEMIAS– ALL / AML / CML
CLASSIFYING CHILDHOOD MALIGNANCY
• BRAIN TUMOURS– Seperate textbook !
• RARE TUMOURS– Thyroid Ca / Melanoma
• EMBRYONAL TUMOURS– Neuroblastoma– Nephroblastoma– Rhabdomyosarcoma– Hepatoblastoma– Retinoblastoma– PNET– Germ Cell Tumour
• BONE TUMOURS– Osteogenic Sarcoma– Ewing’s Sarcoma
• 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)
NEPHROBLASTOMA
• 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
STAGING
• 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 …
DIAGNOSIS AND MANAGEMENT
So good that we’re deintensifying treatment !!
0 50 100 150 200 250 300 350
T ime - months
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cum
ulative Proportion S
urviving
S tage I 94.4% S tage II 96.2% S tage III 84.9% S tage IV 54.2%
Comple te CensoredCh i-squa re = 22 .898 d f = 3 p = 0 .00004
FAVOURABLE HISTOLOGY WILMS’ TUMOUR
• 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%)
NEUROBLASTOMA
• 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
CLINICAL PRESENTATION
• 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
DIAGNOSIS AND MANAGEMENT
• 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
RHABDOMYOSARCOMA
• 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
DIAGNOSIS AND 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
HEPATOBLASTOMA
CLINICAL SCENARIOS IN SOLID PAEDIATRIC
TUMOURS
AN APPROACH FOR THE GENERAL PAEDIATRICIAN
LEUKAEMIA LYMPHOMA or NEUROBLASTOMA
• ASK ABOUT …– bone pain
• WATCH OUT FOR …– swollen gums– lymph nodes– abdominal masses– proptosis
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
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.
1. PANCYTOPAENIA
2. BONE PAIN
• NOT localised
• Wakes the child
• Limp or Reluctance to bear weight
• A toddler who stops walking
• Backache!!
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
• from 6m to 6y …
ABDOMINAL MASSis a tumour until
proven otherwise
ALWAYS CONSIDER SEDATING THE PATIENT IF YOU CAN”T PALPATE THE ABDOMEN !
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
3. ABDOMINAL MASS
No need for
BIOPSY !!
REFER
GERM CELL TUMOUR Feto-protein
BURKITT LYMPHOMA LDH and urate
RHABDOMYOSARCOMA LDH
NEUROBLASTOMA LDH
(3. PELVIC MASS)
No need for
BIOPSY !!
REFER
4. AN UNEXPLAINED MASS
• SITES– Head and Neck– Limbs– Testis
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
7. EYE CHANGES
• White Reflex• Recent Onset Squint• Loss of Vision
RETINOBLASTOMA
• Proptosis
LEUKAEMIA especially Acute Myeloid Leukaemia NEUROBLASTOMA RHABDOMYOSARCOMA
7. EYE CHANGES• Leukocoria
• REFER to an Ophthalmologist urgently for slit lamp examination
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
• Pallor plus Bleeding• Persistent Fever / Apathy / Weight Loss• Bone Pain• Adenopathy• Unexplained Neurological Signs• Unexplained Mass• Eye Changes
IN SUMMARY … REMEMBER THE WARNING SIGNS
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 !