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Solid tumors of Childhood Sariu  Ali: Introduction, CNS Tumors, Neuroblastoma Mohammed Farhan: Neproblastoma, Retinoblastoma, sarcomas, others
Introduction More than 11 million people are diagnosed with cancer anually. <2% occur in children Second commonest cause of death in children (5-14 yo) in developing countries  Adults tumors are epitheilai in orifgin
SOLID TUMORS IN CHILDHOOD  Adrenal medulla & Sympathetic nervous system Bone  Brain Kidney Liver ,[object Object]
Astrocytoma
Glioma
EpendymomaNephroblastoma (Wilm’s tumor) ,[object Object]
OsteosarcomaNeuroblastoma ,[object Object]
Hepatoblastoma,[object Object]
Primary central nervous system (CNS) tumors are second most frequent malignancy in childhood and adolescence.
Mortality among this group approaches 45%.
Generally multimodal Rx. has improved over years due to advances in neurosurgery ,radiation and chemotherapy.,[object Object]
CNS Tumors  Types ,[object Object]
Astrocytomas
Brain stem gliomas
Epandymoma
Optic glioma
PrirmitiveNeuroendocrine  Tumors (PNET)/ Medulloblastoma
Craniopharyngioma
   Pineal region tumors
   Choroid plexus tumors
   germ cell tumors
meningioma,[object Object]
CNS Tumors  Tumor locations  The relative frequency of brain tumor histologic types and the anatomic distribution are shown.
CNS Tumors  Age related differences
CNS Tumors  Clinical presentations   Clinical symptoms depend upon: Age, location, and type of tumor and grade Increased intracranial pressure :secondary to obstruction of CSF at aqueduct :hydrocephalus (infants), headache, papilledema, vomiting
CNS Tumors  WHO classification  ,[object Object]
 >100 histological categories and subtypes
 5 types account for 80%
 juvenile pilocyticastrocytoma
Medullobalstoma/PNET
 Diffuse Astrocytoma
Epandymoma
Craniopharyngioma,[object Object]
occur throughout the CNS
Juvenile pilocyticastrocytoma (JPA) is the most common astrocytoma in children, comprising 20% of all brain tumors. Juvenile pilocyticastrocytoma ,[object Object]

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CNS tumors and Neuroblastomas

  • 1. Solid tumors of Childhood Sariu Ali: Introduction, CNS Tumors, Neuroblastoma Mohammed Farhan: Neproblastoma, Retinoblastoma, sarcomas, others
  • 2. Introduction More than 11 million people are diagnosed with cancer anually. <2% occur in children Second commonest cause of death in children (5-14 yo) in developing countries Adults tumors are epitheilai in orifgin
  • 3.
  • 6.
  • 7.
  • 8.
  • 9. Primary central nervous system (CNS) tumors are second most frequent malignancy in childhood and adolescence.
  • 10. Mortality among this group approaches 45%.
  • 11.
  • 12.
  • 17. PrirmitiveNeuroendocrine Tumors (PNET)/ Medulloblastoma
  • 19. Pineal region tumors
  • 20. Choroid plexus tumors
  • 21. germ cell tumors
  • 22.
  • 23. CNS Tumors Tumor locations The relative frequency of brain tumor histologic types and the anatomic distribution are shown.
  • 24. CNS Tumors Age related differences
  • 25. CNS Tumors Clinical presentations Clinical symptoms depend upon: Age, location, and type of tumor and grade Increased intracranial pressure :secondary to obstruction of CSF at aqueduct :hydrocephalus (infants), headache, papilledema, vomiting
  • 26.
  • 27. >100 histological categories and subtypes
  • 28. 5 types account for 80%
  • 33.
  • 35.
  • 36. the classic site of presentation is the cerebellum
  • 37. The classic neuroradiologic findings of JPA are the presence of an enhancing nodule within the wall of a cystic mass
  • 38.
  • 39. CNS Tumors WHO grading commonly used for astrocytoma The WHO-grading scheme is based on the appearance of certain characteristics: atypia,mitosis, endothelial proliferation and necrosis.
  • 40.
  • 41. PE (careful neurological examination, visual fields and a fundoscopic examination)
  • 43. Serum and CSF ß-HCG, a-FP for germ cell tumors
  • 44. LP: medulloblastoma/PNET, ependymoma, germ cell tumors that spread to the leptomeningesLP contraindicated if newly dx’d hydrocephalus d/t CSF outflow obstruction or infratentorialtumors
  • 45. CNS Tumors Management Treatment – Multimodal
  • 46.
  • 47. Approximately 600 new cases in the U.S. annually
  • 48. Slightly more common in males than females.
  • 49. Median age at diagnosis is approximately 22 months, 89% of new patient diagnoses occurring under the age of 5 years.
  • 50.
  • 51.
  • 52. Cervical/thoracic disease more common in infants <1 yo, adrenal disease more common in >1 yo.
  • 53. In patients less than 1 yo, local tumor only (39%), regional lymphatic spread (18%), metastases (25%), IV-S (18%).
  • 54.
  • 56. 9 months female Failure to thrive Periorbitalecchymosis “Raccoon Eyes” Dysconjugate gaze Dilated fixed pupils T1 MRI extensive mets to base of skull Sphenoid/temporal/zygomatic bones Raccoon eyes caused by obstruction palpebral vessels by mets around eyes Delayed diagnosis: Child abuse/Trauma
  • 57.
  • 63.
  • 67. CATECHOLAMINE METABOLISM TYROSINE Tyrosine Hydroxylase DOPA HOMOVANILLIC ACID (HVA) DOPA Decarboxylase DOPAMINE Monamine oxidase Catechol-O-methyltransferase Dopamine -Hydroxylase NOREPINEPHRINE Phenylethanolamine N-Methyltransferase NB Lack this enzyme VANILLYLMANDELIC ACID (VMA) EPINEPHRINE Catechol- O-Methyltransferase METANEPHRINE
  • 68.
  • 69.
  • 70. Located on distal short arm of chromosome 2.
  • 71. MYCN is a protooncogene normally expressed in developing nervous system and other tissues. MYCN dimerizes with Max, and releases the transcriptional repression mediated by Max homodimers.
  • 72. Amplification usually results in 5-400 copies of the gene, with corresponding increases in protein expression. Co-amplified genes.
  • 73.
  • 74. Neuroblastoma Principle of initial Management Surgery, chemotherapy, and radiotherapy. Depending on the anticipated clinical behavior of the tumor in individual cases considering stage, age, and tumor pathobiology. Surgery Goal: If performed prior to therapy: Establish the diagnosis. Provide tissue for biologic studies. (MYCN, Genomics) Stage the tumor surgically. Attempt to excise the tumor without injury to vital structures. In delayed primary or second-look surgery, the surgeon will determine response to therapy and remove residual disease when possible. Radiation Treatment Neuroblastoma is a radiosensitive tumor. May play role as adjunct to primary therapy with surgery and/or chemotherapy, for palliation of symptomatic lesions, relief of cord compression, or as preparative regimen for BMT.
  • 75. Neuroblastoma Management Low-risk : surgery Intermediate-risk : surgery + chemotherapy ± radiotherapy High-risk: chemotherapy +surgery + radiotherapy
  • 76. References Nelson Text book of Peadiatrics 17th edition Hand book of Hospital peadiatrics 2nd edition Illustrated text book of paediatrics 3rd edition National Cancer unit (US) paediatric oncology unit report CNS malignancies Emed