Presentation is loading. Please wait.

Presentation is loading. Please wait.

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

Similar presentations


Presentation on theme: "PAEDIATRIC SOLID TUMOURS Alan Davidson Haematology - Oncology Red Cross Children’s Hospital."— Presentation transcript:

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

2 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

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 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

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 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”

10 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

11 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

12 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

13 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

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

15 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

16 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

17 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

18 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 excisedGroup II microscopic residua Group III macroscopic residuaGroup IV metastases RHABDOMYOSARCOMA

19 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

20 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

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

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

23  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

24 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

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 from 6m to 6y … ABDOMINAL MASS is a tumour until proven otherwise ALWAYS CONSIDER SEDATING THE PATIENT IF YOU CAN”T PALPATE THE ABDOMEN ! 3. ABDOMINAL MASS

28  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

29  GERM CELL TUMOUR   Feto-protein  BURKITT LYMPHOMA  LDH and urate  RHABDOMYOSARCOMA  LDH  NEUROBLASTOMA  LDH (3. PELVIC MASS) 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 Pallor plus Bleeding Persistent Fever / Apathy / Weight Loss Bone Pain Adenopathy Unexplained Neurological Signs Unexplained Mass Eye Changes IN SUMMARY … REMEMBER THE WARNING SIGNS

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 !


Download ppt "PAEDIATRIC SOLID TUMOURS Alan Davidson Haematology - Oncology Red Cross Children’s Hospital."

Similar presentations


Ads by Google