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pedia - malignancy

med22

QuestionAnswer
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.
Created by: MeanHeem
 

 



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