BRAIN TUMORS IN INFANTS

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1 BRAIN TUMORS IN INFANTS Dr Sergio Valenzuela M.D-( ISPN-ESPN-FLANC)&cols. Head Pediatric Neurosurgery Unit I Instituto de NeurocirugiaAsenjo Santiago CHILE

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3 RATE OF MENINGEAL,BRAIN AND OTHER CNS MALIGNANT TUMORS / ,0 2,5 2,0 1,5 1,0 0,5 0,

4 EPIDEMIOLOGY Incidence of brain tumors in children <1 y.o / % of all intracranial tumors in infants and half on the first 6 months Less than 5% of all cancers in new born children Mortality 50 % between 2 and 9 months old Mainly supratentorial 2/3-1/3 High grades Histology : Astrocitomas-Medulloblastomas- Ependimomas-ATRT-Papillomas-PNET- Teratomas-

5 Rickert,Probst,Gullota Primary intracranial neoplasm of infancy and Early childhood Child s Nerv.Syst.(1997) 13:

6 PEDIATRIC WORLD POPULATION WORLD POPULATION MILLIONS WORLD POPULATION MILLIONS PEDIATRIC 0-14 YEARS OLD % PEDIATRIC 0-14 YEARS OLD % CHILDREN ACTUALLY 1910 MILLIONS BT INCIDENCE 3/ NEW CASES/YEAR

7 SYMPTOMS Evolutive macrocephaly,splitted sutures Frequent vomits (Diferential Dg: GI Reflux ) Irritability/lethargy Seizures Feeding troubles Developmental stop or delay Cerebellar fits Relation w/ prenatal diseases up to 60 %

8 CLINICAL SIGNS Cranial circumference growth Ocular movements disfunction Hemiparesis Oculomotor palsies ( III nerve ) Papilledema Cranial nerves palsies Optic atrophy HIDROCEPHALUS 80 %

9 DIAGNOSIS CLINIC :macrocephaly-vomits-seizures Prenatal-postnatal : ecotomography CT Scan Fetal MRI and post natal Plain skull X rays Delay in diagnosis due to skull elasticity and huge subdural benign collections

10 ETIOLOGICAL FACTORS ESTABLISHED CAUSES HEREDITARY SYNDROMES IONIZING RADIATION HIGH INCIDENCE MATERNAL JOBS (TRANSPORT & AGRICULTURE) DIETS (CURED MEATS-BEER) EXPOSURE TO NITRITES- NOT CONCLUDING BRAIN TRAUMA, ELECTROMAGNETIC FIELDS,PREGNANCY INFECTIONS,TOBACCO ALCOHOL,PESTICIDES

11 TERMINOLOGY CONGENITAL detected at birth or first few days of life* NEONATAL symptoms recognized in the first 12 months* INFANTILE under the age of 2 years old *Boldrey et al J pediatrics 1950

12 DISTRIBUTION IN INFANTS 80 % SUPRATENTORIALS HEMISPHERIC 45 % INTRAVENTRIC % PARASELLAR 12.5 % 20 % INFRATENTORIALS

13 BRAIN TUMORS IN INFANTS GROSS TOTAL REMOVAL STEPS SURGERY BIOPSY + CHEMOREDUCTION + 2 nd LOOK INTENSIVE CHEMO

14 GROSS TOTAL RESECTION

15 BRAIN TUMORS IN INFANTS HYPOTHERMIA HIGH RELATION SURFACE / MASS THIN FAT LAYER ABSENCE OF SHIVERING THERMOGENESIS TROUGH FAT METABOLISM HIGH OXIGEN CONSUMPTION GRAY FAT GETS 10 % OF HEART OUTPUT IN HYPOTHERMIA GETS 75 % HIPOTHERMIA = HYPOXIA = HYPERCAPNIA = ACIDOSIS = HYPOGLICEMIA

16 P.N.E.T. PRE OP

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18 CYSTIC ASTROCITOMA

19 CHOROID PLEXUS PAPILLOMA

20 ANAPLASTIC ST EPENDIMOMA

21 FUNCTION V/S RESECTION OPTIC TRACT GLIOMA WITH PRESERVED VISION CHEMOTHERAPY (PACKER)

22 Five years outcome optic way glioma

23 GERM CELLS TUMOR BIOPSY AFTER 2 CHEMO CYCLES

24 PNET 12 MONTHS POST OP CHEMO BABY RADIOTHERAPY AT 3 YEARS OLD 4 YEARS OLD

25 TUMORES CEREBRALES EN LA INFANCIA D R S E R G I O V A L E N Z U E L A A J E F E D E S E R V I C I O N C P E D I A T R I C A I N S T I T U T O D E N E U R O C I R U G Í A A S E N J O

26 MEDULLOBLATOMA

27 MEDULLOBLASTOMA

28 MEDULLOBLASTOMA

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30 Medulloblastoma Post op with remanent and metastases Post Chemo

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33 11 months old Surgery Chemo Rdt at 3 y.o. 5 years surv.

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35 ATYPICAL TERATOID RABDOID TUMOR IN INFANTS 6 Months old-subtotal resection-intensive chemorecurrence at 11 months-new surgery-death at 14m.o

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40 BRAIN TUMORS IN CHILDREN UNDER 1 YEAR OLD CONGENITAL & NEONATAL ASENJO S NEUROSURGICAL PEDIATRIC DEPARTMENT SERIES (30 YEARS) 105 CASES MORTALITY 41 %

41 INFANTS UNDER 1 YEAR OLD OPERATED /YEAR

42 GLOBAL DISTRIBUTION N:105 ACCORDING TO WHO 2007 HYSTOLOGICAL CLASIFICATION

43 LOCALIZATION

44 Astrociytoma 24 Craneofaring. 0 Ependymoma 47 Fibrosarcoma 0 Ganglioglioma 0 Hamartoma 0 Hystiocitosis 0 GLOBAL MORTALITY Menigeal gliomatosis 100 Medulloblast. 82 Neuroblastoma 100 Plexiform neurofibroma 0 Plexus papilloma 16 PNET 50 Rhabdomiosarcoma 100 ATRT 60 Teratoma 50

45 TREATMENTS- OUTCOMES ACTUAL THERAPIES GTR + CHEMO RADIOTHERAPY AT 3 YEARS OLD OUTCOME AT 5 YEARS FREE OF DISEASE Choroid plexus Papilloma plexos 100 % Astrocitoma 76 % PNET 50 % Medulloblastoma 18 % ATRT 40 %

46 RADIOSURGERY IN PEDIATRICS?? When?? Initial therapy in remanents Recurrences At 2 years old?? Before???? WHAT TYPE???? Conformational? Gamma knife?? Cyber knife??

47 Learning Psychologic Growth Hearing More get better but they face complications Second Tumor Infertility Hormones

48 TO REMEMBER Brain tumors in infants are bad...but no so bad Most of them are ST and low grades Take care of hydrocephalus-etv-evd shunt GTR is the gold standard-very careful Two steps surgery in difficult cases Chemo helps according to molecular biology Rdt. under consideration in selected cases Watch intraoperative bleeding and hypothermia Brain is developing late outcome neurorehab.

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