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pedia - malignancy
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| Question | Answer |
|---|---|
| What is the definition of Leukemia? | Leukemia is a cancer that starts in the bone-forming tissue (e.g., bone marrow), causing abnormal blood cell production. |
| What is the mean age of diagnosis for Leukemia in pediatrics? | 2-6 years. |
| List the three main types of childhood leukemia by frequency (highest to lowest). | Acute Lymphocytic Leukemia (ALL) (80%), Acute Myeloid Leukemia (AML) (15%), and Chronic Myeloid Leukemia (CML) (less than 5%). |
| List four common general clinical features of Leukemia. | Fever, fatigue, weight loss, bruising, bone pain, and easy bleeding. |
| What is the mechanism by which leukemia causes anemia, neutropenia, and thrombocytopenia? | Infiltration of leukemic cells into the bone marrow causes bone marrow failure. |
| Acute Lymphocytic Leukemia (ALL) accounts for what percentage of all pediatric cancers? | 30%. |
| List three genetic syndromes or conditions associated with an increased risk of ALL. | Down syndrome, Severe Combined Immunodeficiency (SCID), and Fanconi anemia (congenital bone marrow failure). |
| What are the two most common presenting clinical features of Acute Lymphocytic Leukemia (ALL)? | Fever and bone or joint pain (often causing refusal to bear weight). |
| Which ALL immunophenotype is most common, and what is its frequency? | Precursor B-cell phenotype, accounting for 70-80% of cases. |
| What is the prognostic significance of a White Blood Cell (WBC) count that is greater than 50,000 cells/mm3 at the time of ALL diagnosis? | It is considered an unfavorable prognostic factor. |
| What is the prognostic significance of a child being between 1 and 9 years old at the time of ALL diagnosis? | It is considered a favorable prognostic factor. |
| What most common finding on a Complete Blood Count (CBC) that suggests a diagnosis of ALL? | Anemia and thrombocytopenia. |
| What is required for the definitive confirmation of Acute Lymphocytic Leukemia (ALL)? | Bone marrow evaluation demonstrating replacement of marrow by lymphoblasts. |
| What type of involvement must be determined by obtaining spinal fluid (CSF) in the workup of ALL? | Central Nervous System (CNS) involvement. |
| What is the significance of Hyperploidy (more than 53 chromosomes) in ALL? | It is a favorable prognostic factor. |
| What organ involvement at diagnosis is considered an unfavorable prognostic factor for ALL? | Organomegaly, Central Nervous System (CNS) involvement, or a mediastinal mass. |
| What is the most common sign of leukemic cell infiltration into tissues? | Lymphadenopathy, hepatosplenomegaly, CNS manifestations, or testicular disease. |
| What is the female sex considered in terms of ALL prognosis? | A favorable prognostic factor. |
| What is the classification of Acute Lymphocytic Leukemia based upon? | The cell of origin, immunophenotype, and cytogenetic characteristics of the leukemic cells (i.e., lymphoblasts). |
| What is the most common ALL subtype among the Pre-B-cell phenotype? | CALLA-positive (70% of Pre-B-cell ALL). |
| What WBC count is considered a favorable prognostic factor for ALL? | A WBC count less than 50,000 cells/mm3. |
| What is the prevalence of ALL in all childhood leukemias? | 80-85%. |
| What are the three main categories of pediatric oncology covered in the lecture, besides Leukemia? | Lymphomas, Brain Tumors, and Renal and Suprarenal Tumors. |
| What is the general rule regarding back pain in children in the context of malignancy? | Back pain in children must always be investigated. |
| What is the classification (Ploidy) considered unfavorable for ALL prognosis? | Low ploidy (fewer than 53 chromosomes within leukemic cells). |
| What is the most common presentation of Hodgkin Lymphoma (HL)? | Painless, firm, non-tender enlargement of the lymph nodes, most commonly in the cervical or supraclavicular region. |
| What is the characteristic cell type found in Hodgkin Lymphoma? | Reed-Sternberg (RS) cells. |
| What are the three "B" symptoms associated with poorer prognosis in Lymphoma? | Fever, drenching night sweats, and unexplained weight loss (more than 10% of body weight over 6 months). |
| What are the four main subtypes of Non-Hodgkin Lymphoma (NHL)? | Burkitt Lymphoma, Lymphoblastic Lymphoma, Large B-Cell Lymphoma, and Anaplastic Large Cell Lymphoma (ALCL). |
| Which type of NHL is considered the fastest growing tumor in humans? | Burkitt Lymphoma. |
| What is the characteristic histological appearance of Burkitt Lymphoma? | Starry-sky pattern. |
| What is the most common type of solid tumor in pediatrics? | Brain and Central Nervous System (CNS) tumors. |
| What are the two most common presenting symptoms of a pediatric brain tumor? | Headache and vomiting (often worse in the morning). |
| What clinical finding is highly suspicious for a posterior fossa tumor? | Ataxia and hydrocephalus (leading to signs of increased Intracranial Pressure - ICP). |
| What is the definitive diagnostic procedure for a brain tumor? | Neuroimaging (MRI) followed by biopsy. |
| What is the most common abdominal malignancy in children? | Wilms Tumor (Nephroblastoma). |
| What is the peak age of incidence for Wilms Tumor? | 2-5 years. |
| What is the classic clinical presentation of Wilms Tumor? | Painless abdominal mass. |
| What genetic syndrome is associated with Wilms Tumor, Aniridia, Genitourinary anomalies, and intellectual disability (WAGR syndrome)? | Deletion of the WT1 gene on chromosome 11. |
| What is the most common extracranial solid tumor in infancy? | Neuroblastoma. |
| Where does Neuroblastoma commonly originate? | The adrenal medulla or sympathetic ganglia. |
| What lab markers (urinary catecholamine metabolites) are elevated in over 90% of children with Neuroblastoma? | Vanillylmandelic acid (VMA) and Homovanillic acid (HVA). |
| What is the definition of Tumor Lysis Syndrome (TLS)? | A metabolic derangement caused by the rapid death of malignant cells after chemotherapy, leading to the release of intracellular contents. |
| What are the four key metabolic abnormalities seen in Tumor Lysis Syndrome (TLS)? | Hyperuricemia, Hyperkalemia, Hyperphosphatemia, and secondary Hypocalcemia. |
| What is the most common cause of morbidity and mortality in TLS? | Acute kidney injury (from uric acid and calcium-phosphate crystals) and cardiac arrhythmias (from hyperkalemia). |
| What is the primary management strategy for the prevention of TLS? | Aggressive IV hydration and use of Allopurinol or Rasburicase (for hyperuricemia). |
| What is Superior Vena Cava (SVC) Syndrome? | Compression or obstruction of the SVC, often by a mediastinal mass (e.g., T-cell Lymphoblastic Lymphoma or Hodgkin's disease). |
| What are the signs and symptoms of SVC Syndrome? | Facial and upper extremity swelling, dyspnea, plethora (flushing/redness), and dilated veins on the chest and neck. |
| What is the immediate management priority for a patient with suspected SVC Syndrome? | Airway management and urgent initiation of treatment (chemotherapy/radiation) to shrink the tumor. |
| What is the most common cause of Spinal Cord Compression (SCC) in pediatric malignancy? | Epidural tumor invasion (most commonly from Neuroblastoma or Sarcomas). |
| What is the cardinal symptom of Spinal Cord Compression? | Acute or progressive back pain (must always be investigated in children). |
| What is the immediate management for suspected Spinal Cord Compression? | High-dose steroids and urgent neuroimaging (MRI) to confirm the site and extent of compression, followed by radiation or surgery. |