childhood-tumors-powerpoint-pedias-.pptx

tumor pada anak

1. hepatoblastoma By: Brittni McClellan

2. Hepatoblastoma description Description: Most common form of liver cancer in children. Tumor Marker: AFP Can also secrete b- hCG Pathophysiology: Originate from Immature Liver Precursor Cells Genetics: Loss of Heterozygosity of the Chromosome Arm 11p. Associated with Beckwith-Wiedemann Syndrome (BWS)

3. Hepatoblastoma – H&P Signs and Symptoms: Asymptomatic Abdominal Mass Isosexual Precocity: Penile/Testicular Enlargement without Pubic Hair Anorexia Talipes Equinovarus Persistent Ductus Arteriosus Tetralogy of Fallot Extrahepatic Biliary Atresia Renal Anomalies

4. Hepatoblastoma - Diagnosis Diagnosis: CBC: Normochromic Normocytic Anemia Thrombocytosis Other Labs Liver Enzyme Levels Elevated in 15-30% AFP As high as 100,000-300,000 Biopsy: Open Biopsy or Complete Surgical Resection Imaging: Radiography: Mass in RUQ of Abdomen U/S: CT: Reveals involvement of nearby structures Reveals if Pulmonary Metastases are present MRI: Superior to CT if CT is inconclusive. PET: Follow-Up Evaluation Radionuclide Bone Scanning: Reveals Bone Metastases

5. Hepatoblastoma – treatment Medication: Cisplatin: Most active single agent Cisplatin/5-FU/Vincristine Combo Radiotherapy Resection/Transplantation

6. Neuroblastoma description Description: Most Common Extracranial Solid Tumor of Childhood Most Common Malignancy of Infancy Pathophysiology: Derived from Neural Crest Cells Arises anywhere along the Sympathetic Chain of the Peripheral Nervous System Most Common: Adrenal Medulla Genetics : Due to Inherited Mutations in the ALK Tyrosine Kinase Associated with N-MYC Oncogene Associated with Congenital Central Hypoventilation Syndrome and PHOX2B Mutations. Small Round Blue Cell Tumor Homer Wright Rosettes Bombesin +

7. neuroblastoma – H&P History: General Appearance, Activity Level, and Appetite Based on Tumor Location: Thoracic: Chest Pain, Cough, Respiratory Distress Abdominal: Pain, Swelling Bone Marrow: Fatigue (Anemia) Associated Conditions Neurofibromatosis Type I Hirschsprung Disease Central Congenital Hypoventilation Syndrome Signs and Symptoms: Based on Location: Abdomen: Firm, Fixed, Irregular Mass Abdominal Distention Signs of Bowel Obstruction Hypertension (RAS) Genital/Lower Extremity Edema (Lymphatic Obstruction) Cervical/Thoracic Mass: Respiratory Distress Horner Syndrome Superior Vena Cava Syndrome Paraspinal Mass: Nerve Root Compression Metastatic Disease Symptoms Bone Metastases (Pain) Bony Orbit

8. neuroblastoma – H&P

9. neuroblastoma - Diagnosis Diagnosis: CBC: Decreased Hemoglobin, Platelets, and/or WBC indicate Bone Marrow Involvement Urine Catecholamine Metabolites are Elevated: Homovanillic Acid (HVA) Vanillylmandelic Acid (VMA) Imaging U/S or CT: Find Primary Tumor Tumor is CALCIFIED MIBG Scan: Taken up by 90% of Neuroblastomas Can detect bone/soft tissue involvement Bone Scan: Find Bone Mets

10. neuroblastoma – treatment Medication Vincristine, Doxorubicin, Cyclophosphamide, Cisplatin, Carboplatin. Etoposide, Topotecan 13-cis-Retinoic Acid: Induces differentiation of Neuroblastoma Cells and Improves Survival Immunotherapy : Antibodies against GD2 Possibility of Spontaneous Regression

11. retinoblastoma description Description: Most Common Primary Intraocular Tumor Malignant Tumor of the Embryonic Neural Retina Pathophysiology: Loss of function of both copies of the RB1 gene Located on Chromosome 13 Spreads via the Optic Nerve Vitreous Seeding: Tumor Cells Break Off Two Forms: Hereditary Retinoblastoma 45% of all RB 1 RB Gene is Dysfunctional in all cells Non-Hereditary Retinoblastoma 55% of all RB 2 acquired mutations must occur in a single retinal cell.

12. retinoblastoma – H&P Patient History: Family History Leukocoria (White Pupil) Strabismus Rare: Pain, Inflammation, Vision Problems Metastatic Symptoms: Neurological Signs, Orbital Masses, Bone Pain, Anorexia, Cytopenia Associated Conditions: 13q Deletion Syndrome RB, Dysmorphism, Mental Retardation, GU Anomalies High Risk of Osteosarcoma in the Future Physical Exam: Only 3% discovered on routine fundoscopic examination Leukocoria/Strabismus Check for Red Reflex in Darkened Room Screen for RB in Office Evaluate Anisocoria Unequal Pupils

13. retinoblastoma - Diagnosis Diagnosis: Ophthalmologic Examination By a specialist in pediatric ocular tumors Biopsy: Biopsy confirmation is RARE. Risk of seeding CBC: Asses for Bone Marrow involvement CSF Cytology Evaluation of Leptomeningeal Spread Chromosome Analysis Only 5% are detectable, but should be done anyways. Imaging: CT/MRI: Evaluation of Primary Tumor Evaluation of any metastases.

14. retinoblastoma – treatment Medication Eradicate Tumor/Eye Salvage Therapy Plaque Radiotherapy: Radioactive seeds sewn into episcleral surface about RB lesion. Provides local radiation to the tumor External Beam Radiation Therapy Photocoagulation: Cryotherapy Locally Delivered Chemotherapy Systemic Chemotherapy Tumor Resection with Enucleation

15. Wilms tumor (Nephroblastoma) description Description: Malignant Tumor of the Kidney Also called Nephroblastoma Pathophysiology: 10-20% Hereditary Associated with WAGR, Beckwith-Wiedemann, and Denys- Drash Syndromes Tumor Suppressor Gene: WT1 on Chromosome 11p13 WT2 on Chromosome 11p15

16. Wilms tumor – H&P Patient History: Family History Abdominal Distention/Pain Hematuria Fever, Anorexia, Vomiting Rapid Increase in Abdominal Size Physical Exam: Asymptomatic Abdominal Mass Extends from Flank Towards Midline Anemia Fever Hypertension Varicocele Aniridia, Hemihypertrophy, Cryptorchidism, Hypospadias Proteinuria/Hematuria

17. Wilms tumor - Diagnosis Diagnosis: Labs: CBC, Electrolytes, Urine Analysis, Liver and Kidney Function Tests, Coagulation Factors Imaging: U/S: Diagnostic of Renal Mass Evaluations extention into Inferior Vena Cava CT Scan: Evaluate for Metastases Bone Scan: Evaluate for Metastases Pathology: CT/MRI: Gross: Cystic with hemorrhages and necrosis. No calcification May extend into Inferior Vena Cava Histology: Triphasic Pattern: Blastemal, Epithelial, and Stroma Cells Blastemal Cells aggregate in nodules like primitive glomeruli The presence of diffuse anaplasia indicates poor prognosis.

18. Wilms tumor – treatment Medication For stages I and II: Vincristine and Actinomycin D every 3 weeks for 6 months For stages III and IV: Vincristine, Actinomycin D, and Doxorubicin for 6–15 months Add cyclophosphamide and/or Etoposide for higher-stage anaplastic tumors (stage IV focal or II–IV diffuse). Nephrectomy Radiotherapy

19. Neuroblastoma vs wilms tumor

20. Question 1 A 2-year-old girl is brought to the office by her parents after blood was noticed in her urine. The parents say the patient has had intermittent abdominal pain during the past two months but has been otherwise well. On physical examination, the abdomen is slightly distended and a mass is palpated in the right upper quadrant. Results of urinalysis are positive for blood and protein. Which of the following is the most likely diagnosis? Cystic nephroma Cystitis Mesoblastic nephroma Neuroblastoma Wilms tumor e) Wilms tumor

21. Question 2 A 3-year-old boy is found to have an abnormal red reflex in his left eye on routine examination. Funduscopic examination shows a proliferative retinal mass. After surgical removal of the specimen, pathologic examination reveals small, round cells with hyperchromatic nuclei, some rosette arrangement and isolated regions of necrosis. There is no involvement of the optic nerve or tumor extension into adjacent brain tissue. The patient undergoes eye enucleation without surgical complications.  As the child ages, which of the following disorders is the patient also at risk for developing? Acoustic neuroma. Colon cancer. Neurofibroma. Osteosarcoma. Wilms tumor. d) Osteosarcoma