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By: Cecilia Galan- Fernandez, DPPS, DPSHBT, DPSPO Place: 5 th floor ASMPH Date : January 11,2011

SOLID. By: Cecilia Galan- Fernandez, DPPS, DPSHBT, DPSPO Place: 5 th floor ASMPH Date : January 11,2011. CANCER IN CHILDREN. Cancer Pathogenesis. All kinds of cancer, including childhood cancer, have a common disease process cells grow out of control, develop abnormal sizes and

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By: Cecilia Galan- Fernandez, DPPS, DPSHBT, DPSPO Place: 5 th floor ASMPH Date : January 11,2011

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  1. SOLID By: CeciliaGalan- Fernandez, DPPS, DPSHBT, DPSPOPlace: 5th floor ASMPHDate : January 11,2011 CANCER IN CHILDREN

  2. Cancer Pathogenesis All kinds of cancer, including childhood cancer, have a common disease process cells grow out of control, develop abnormal sizes and shapes ignore their typical boundaries inside the body destroy their neighbor cells spread (or metastasize) to other organs & tissues As cancer cells grow, they demand more nutrition from the body Normally, your body forms new cells as you need them, replacing old cells that die Cell cycle

  3. Epidemiology Incidence 14/100,000 Among all age groups, the most common childhood cancers are leukemia, lymphoma, and brain cancer. As children enter their teen years, there is an increase in the incidence of osteosarcoma (bone cancer). The sites of cancer are different for each type

  4. Risk Factors Pediatric vs. Adult Cancers Lifestyle factors that contribute to the development of cancer in adults: smoking diet obesity occupation prolonged exposure to carcinogenic agents

  5. For the 0-14 yrs old Acute leukemias (ALL-most common) Central nervous system tumors Neuroblastoma Wilms’ tumor Non Hodgkin’s Lymphoma Rhabdomyosarcoma, soft tissue tumors,germ cell tumors, retinoblastoma and bone tumors For 15 – 19 yrs old Hodgkin’s disease Germ cell tumors Osteosarcoma, Ewing’s sarcoma

  6. Risk Factors Pediatric vs. Adult Cancers Lifestyle factors are not associated with cancer in children Genetic condition- anomalies Family history Environmental- ionizing radiation, chemicals Infections – EBV Chemotherapy Precise cause – unknown

  7. Signs of Childhood Cancer Continued, unexplained weight lossHeadaches, often with early morning vomitingIncreased swelling or persistent pain in bones, joints, back, or legsLump or mass, esp. in the abdomen, neck, chest, pelvis, or armpitsDevelopment of excessive bruising, bleeding, or rash Constant/recurrent infectionsA whitish color behind the pupilNausea which persists or vomiting with or w/o seizure Constant tiredness or noticeable palenessEye or vision changes which occur suddenly and persistsRecurrent or persistent fevers of unknown origin

  8. WHY DIFFICULT TO DIAGNOSE CHILDHOOD CANCER? • Vague manifestations • Childhood malignancies are rare • Doctor’s reluctance to consider cancer diagnosis

  9. Important Factors to Cure • EARLY DETECTION • PROPER TREATMENT • GOOD SUPPORTIVE CARE

  10. RETINOBLASTOMA

  11. RETINOBLASTOMA Most common intraocular malignancy of childhood • 2nd most freq solid malignancy • 80% are diagnosed before 3-4 years old • Median age at diagnosis: 2 years old • Beyond 6 years old is rare!!! • Incidence: 1. 60% --non-heriditary & unilateral 2. 25% --hereditary & bilateral 3. 15% --hereditary & unilateral

  12. CLASSIFICATION 1.Laterality ---unilateral or bilateral 2.Focality ---Unifocal or multifocal 3.Genetics --- hereditary or nonheriditary

  13. UNILATERAL -Knudson’s two hit phenomenon -15% carry constitutional mutation BILATERAL -asstd. with advanced parental age -present earlier than unilateral -hereditary form -worse prognosis -autosomal dominant LATERALITY

  14. Retinoblastoma RB gene on Chromosome 13, “two-hit theory” Round blue cell tumor arising in any of the nucleated layers of the retina Hematogenous, lymphatic spread, direct extension thru the optic nerve Only 10% detected by ophthalmologic screening

  15. COMMON SIGNS & SYMPTOMS • Leukokoria --most common • Strabismus --2nd most common • Decreased visual acuity • Inflammatory changes • Glaucoma • Vitreous hemorrhage resulting in black pupil • Rubeosisiridis –(neovascularization of surface of iris) *in 50% with advanced dsespontaneous bleed---hyphema

  16. Investigations for Diagnosis of Retinoblastoma 1.Examinations -done under anesthesia by pedia ophtha -done by pedia oncologist -Audiology evaluation if chemo with carbplatin 2. Imaging studies -CT scan of brain and orbits 3. Laboratory evaluations -CBC,Bld chem,electrolytes -Creatinine clearance if chemo with carboplatin

  17. Investigations for Diagnosis of Retinoblastoma 4. Diagnostic Studies -Lumbar puncture ---only if with radiographic or clinical suspicion of CNS disease -Bone scan --- only if with bone pain or other extra- ocular disease -Bone marrow biopsy –only with abnormal CBC(without alternative explanation) or other extraocular disease 5.Pathologic evaluation---if enucleation is performed

  18. Histology: The only human tumor that is radically treated without tissue biopsy confirmation Flexner-Wintersteiner rosettes: lined by tall cuboidal cells that circumbscribe and an apical lumen

  19. Patterns of SpreadIntraocular:1. With endophytic growth, there is a white hazy mass.2. With exophytic growth, there is retinal detachment.3. Most tumors have combined growth.4. Retinal cells frequently break off from the main mass and seed the vitreous or new locations on the retina.

  20. Extraocular: Retinoblastoma spreads first to surrounding structures and then by hematogenous or lymphatic extension. Retinoblastoma invades the optic nerve. From there it can spread directly along the axons to the brain or may cross into the subarachnoid space and spread via the CSF to the brain. Hematogenous spread leads to metastatic disease, most commonly to brain, bone marrow, or bone. Lymphatic spread is rare because there is minimal lymphatic drainage of the orbit. Occasionally, retinoblastoma spreads lymphatically to the preauricular and submandibular nodes.

  21. PARTS OF THE EYE

  22. retinoblastoma

  23. SITES OF METASTASIS • BRAIN • BONE • BONE MARROW • LUNGS

  24. RISKS FOR METASTATIC SPREAD 1. If optic nerve stump is <5mm—bad >5mm---better 2. Tumor invasion into the anterior chamber 3. Large tumor with vitreous seeding 4. Rubeosisiridis 5. Glaucoma

  25. Retinoblastoma GOAL OF TREATMENT: Primary Goal: Cure!!!! Secondary Goal: Preservation of vision

  26. TREATMENT • Surgery Enucleation—if no hope for salvage of useful vision II. Radiotherapy III. Chemotherapy

  27. Neuroblastoma Most common extracranial solid tumor in infancy 8-10% of all childhood malignancies Affects infants & preschool 50% younger than 2 year old 30% younger than 1 year old

  28. NEONATAL NEUROBLASTOMA • Subcutaneous nodules. If massaged, a zone of pallor develops around the nodule due to catecholamine discharge • Extensive metastasis to the liver • Bone marrow involvement • Massive adrenal hemorrhage into tumor • Hydropsfetalis and/or signs of erythroblastosisfetalis • Stage 4S • Diagnosis: prenatal ULTZ • In many cases, a mass is not palpable • Only 50% have elevated urinary catecholamines • Prognosis: favorable course (if with + favorable factors) • Treatment: Curative by surgery • Course: Others regress

  29. NEUROBLASTOMA • Originates from primordial neural crest cells that normally give rise to adrenal & sympathetic ganglia • Are tumors of the sympathetic nerve tissue • Can occur anywhere along the sympathetic neural pathway

  30. Neuroblastoma – Clinical Features Arise from the sympathetic outflow Primary sites 70% abdominal primary (50% adrenal medulla, 50% extra-adrenal tissue) Thoracic tumours, posterior mediastinum (20%) Head & neck tumours (10%) Epidural (dumbbell or hourglass shaped) tumors – cord compression/paraplegia Bone Lymph nodes are enlarged Lungs—rarely involved(0.7%), involvement should be proven by bx Pelvis---constipation,urinaryretention,presacral

  31. MANIFESTIONS • Excessive catecholamine (VMA/HVA) • intermittent attacks of sweating,pallor • Headaches,palpitation • Hypertension—renin induced due to renovascular compromise & seen in 1-5%

  32. MANIFESTATIONS 2. Signs & symptoms of Vasoactive Intestinal Peptide (VIP) • Intractable watery diarrhea resulting to failure to thrive • Abdominal distention • hypokalemia

  33. MANIFESTATIONS 3. Acute myoclonic encephalopathy • Bursts of rapid involuntary random eye movements in all directions of gaze (OPSOCLONUS) • Motor incoordination due to frequent,irregular jerking of muscles of the limbs and trunk (MYOCLONIC JERKING) Note: 1.May or may not resolve after the tumor is removed or symptoms may resolve only after several months. Some , it may be permanent 2. Prognosis is good

  34. PATHOPHYSIOLOGY OF OM • NOT DUE due to direct involvement of the CNS by tumor or due to the production of catecholamines • It is associated by well-defined IgG & IgM autoantibodies that bind to the cytoplasm of cerebellar Purkinje cells & to some axons in the white matter • Diffuse & extensive lymphocytic infiltration with lymphoid follicles is a characteristic histologic feature of OM • This suggests an IMMUNE-MEDIATED MECHANISM OF THISRARE SYNDROME

  35. Recommended Criteria at INSS Conference for a Diagnosisof Neuroblastoma • Established if: • (1) Unequivocal pathologic diagnosisa is made from tumor tissue by light microscopy (with or without immunohistology, electron microscopy), and/or increased urine or • serum catecholamines or metabolites.b OR • (2) Bone marrow aspirate or trephine biopsy contains unequivocal tumor cellsa (e.g.,syncytia or immunocytologically positive clumps of cells) and increased urine or • serum catecholamines or metabolites Source: Manual of Pediatric Hematology & Oncology (4th ed) p534

  36. Recommended Studies for Assessment of Extent of Disease Tumor siteRecommended tests Primary site CT and/or MRI scana with 3-D measurements; MIBG scan.b Metastatic sites Bone marrow Bilateral posterior iliac crest marrow aspirates and trephine core bone marrow biopsies required to exclude marrow involvement. A single positive site documents marrow involvement. Core biopsies must contain at least 1 cm of marrow (excluding cartilage) to be considered adequate. Bone MIBG scan; 99Tc scan required if MIBG scan negative or unavailable. Plain radiographs of positive lesions. Lymph nodes Clinical examination (palpable nodes confirmedhistologically. CT scan for nonpalpable nodes (3-D measurements). Abdomen/liver CT and/or MRI scana with 3-D measurements. Chest AP and lateral chest radiographs. CT/MRI necessary if chest radiograph positive, or if abdominal mass/nodes extend into chest

  37. NB – Clinical features Metastases Bone marrow- anaemia Bone (Pain) Orbit (Peri-orbital Skin (blue-berry muffin)

  38. NB – Therapy Surgery –laminectomy to decompression Chemotherapy-responsive in 80-85%;advantageous in making surgery safer by reduction of tumor size Transplant(Stage 4, Nmyc amplification HD Chemo with Autologous Bone Marrow Transplant

  39. Evaluation of a Lymph Node • A diagnosis of lymphoma or any malignancy must always be considered when evaluating a child with an enlarged peripheral lymph node • It must be emphasized that not all palpable nodes are pathologically enlarged and that most pathologically enlarged nodes are benign. • Points to consider in evaluating a lymph node: I. LOCATION OF THE NODE a. Nodes of the axilla, neck and groin are often palpable in normal children b. Palpable supraclavicular nodes -- always considered abnormal * if with left-sided (Virchow’s) – metastases from intra-abdominal CA * if with right-sided nodes --- intrathoracic disease

  40. IIWhen size matters • a.Cervical and axillary nodes --- > 1 cm • b. Inguinal node ------ >1.5 cm • c. Epitrochlear node ----> 0.5 cm

  41. IV.INDICATIONS III. GENERALIZED OR LOCALIZED? a. Generalized --- involvement of more than two (2) noncontiguous areas caused by many different disease process b. Localized ---- enlarged lymph nodes within contiguous anatomic regions and usually due to one of two ( infection or malignancy) III. CHARACTER Malignant nodes ---- non-tender, firm, rubbery and rapidly enlarging becoming cnonfluent and located in the supraclavicular region

  42. INDICATIONS FOR LYMPH NODE BIOPSY • Chronic,persistent,progressive adenopathy • If an infectious etiology has not been uncovered, a dominant node that persists for 6 weeks should be biopsied . • Node > 2.5 cm in diameter in the absence of signs of infection that warrant a trial of antibiotic • Supraclavicular adenopathy • Sytemic symptoms

  43. Non-Hodgkin Lymphoma • 5-7% malignant disease of childhood • 3rd most common childhood malignancy • 60% of childhood lymphomas • M>F • Peak age: 5-15 years old • Etiology: unknown • Risk factors: • Genetic-- SCID, WAS • Posttransplantationimmunosuppression • Drugs ex. Phenytoin (cause pseudolymphoma that regress with cessation of treatment) • Radiation – treatment with radiotherapy (4-5% develop NHL within 10 years • Virus---EBV and HIV

  44. APPROACH TO THE DIAGNOSIS OF NON HODGKIN’S LYMPHOMA • History and PE childhood lymphoma more often extranodal rapid tumor growth frequently involved site: Intra-abdominal ( B-cell) Intrathoracic (precursor T-cell) Sites: Abdomen --- primary site (35%) Head and neck ---- 13% Mediastinum ---- 26% CNS and BM --- CNS involvement and epidural tumor uncommon at presentation but more common in the presence of BM involvement. --- 2/3 with BM disease have simultaneous CNS involvement --- Intracranial tumor more likely epidural although it can occur within brain parencyma

  45. B SYMPTOMS OF HODGKIN LYMPHOMA 1. Unexplained fever with temp above 38.0ْ C orally 2. Drenching night sweats 3. Unexplained weight loss of 10% within 6 months preceding diagnosis Source: Principles & Practice of Pediatric Oncology 5th ed p.701

  46. IMPORTANT INVESTIGATIONSFOR NHL • Histologic analysis- Primary mode of definitive diagnosis • CBC and APC • Chemistries and Serum electrolytes • Liver and renal function tests • SLDH- measures tumor volume • Uric acid • Imaging studies • Chest X-ray • Chest CT • Abdominal CT/ Ultrasound • BMA with biopsy ( bilateral) • CSF examination -- essential

  47. ST. JUDE CHILDREN’S STAGING SYSTEM STAGE I • A single tumor (extranodal) or single anatomic area (nodal) with the exclusion of mediastinum or abdomen STAGE II • A single tumor (extranodal) with regional node involvement • Two or more nodal areas on the same side of the diaphragm • Two single (extranodal) tumors with or without regional node involvement on the same side of the diaghragm • A primary gastrointestinal tract tumor, usually in the ileocecalarea, with or without involvement of associated mesenteric nodes only STAGE III • Two single tumors (extranodal) on opposite sides of the diaphragm • Two or more nodal areas above and below the diaphragm • All the primary intrathoracic tumors (mediastinal, pleural and thymic) • All extensive primary intraabdominal disease • All paraspinal or epidural tumors, regardless of other tumor site(s) STAGE IV • Any of the above with initial CNS and/or bone marrow involvement

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