CPHON Questions and Answers

CPHON Questions and Answers Auer rods AML distinguishing feature Leukemia Malignant disorder of the blood and blood forming organs including the bone marrow, lymph nodes and spleen Cytogenetics for Leukemia Ploidy; abnormalities found in more than 90% of patients with ALL; with number of chromosomes and structures (translocation) Hyperdiploidy in ALL More than 46; favorable if not more than 50 Genetic associations with leukocytes Trisomy 21, schwachman diamond syndrome, neurofibromatosis, fanconi anemia, Bloom syndrome, ataxia telanflectasia Favorable AML Age 2, WBC 50K, female, absence of extramedullary disease, BMI between 11&94th percentile, Down syndrome Favorable ALL Age 2-10 at diagnosis, WBC 50k, neg MRD at day 29 MRD Ratio of leukemia cells to normal bone marrow cells; use DNA-PCR probes; elevated = poor prognosis and increased risk of relapse Goal is 5% blasts Consolidation in leukemia chemo Peg, MTX, VCR, doxo, corticosteroid, cytarabine, oral MTX, 6MP Maintenance Chemo in leukemia 2-3 years 6MP, MTX, VCR, corticosteroid, CNS prophylaxis Classifications for Leukemia French American British and WHO Immunophenotyping Monoclonal antibodies that develop in response to changes in the expression of cell surface antigens: cd10 positive in 80% of patients with ALL ALL Most common risk for TLS AML Most common risk for DIC Adolescents Most common age group to be dx with lymphoma Non-Hodgkin's Lymphoma Originates in cells and organs of immune system, more systemic, spreads rapidly and unpredictably and often aggressively, lymphoid lineage Non-Hodgkin's Lymphoma risk Increased risk with wiskotts aldrich syndrome, ataxia telanfiectisa, post BMT, SCIDS, AIDS, other immunosuppressive disorders EBV Role unclear but thought to precede or occur with b-cell transformation with Burkitts 4 types of Non hodgkins Precursor (lymphoblastic), Burkitts, diffuse large B cell, anaplastic large cell Induction chemo for AML 3-7 days of cytarabine, doxo, 6tg, prednisone, vp16 ; abx and antifungal prophylaxis Diploid 46 chromosomes Staging for non hodgkins Ann Arbor or Murphy (st Jude) Good prognostic for non hodgkins Primary tumor head/neck/LN/GI stage 1 or 2 Poor prognostic for non hodgkins Incomplete remission, increased tumor burden, LDH 1000, dealt in treatment Hodgkin's lymphoma Involves lymphatic system, including spleen and all lymph nodes; characterized by reed sternburg cells Reed-Sternberg cells Abundant cytoplasm and large nucleus; most of tumor composed of inflammatory cells and fibrosis due to cytokines release Night sweats Cardinal sign of lymphoma Hodgkin's lymphoma B cell sxs Fatigue, fever for more than 3 days, weight loss 10% in 6mo, night sweats Favorable prognostic of hodgkins Localized involvement, abscense of B sxs and bulky disease Unfavorable hodgkins Advanced stage, B sxs, bulky disease, extranodal disease, male, elevated ESR Astrocytomas Most common brain tumor ~50% Astrocytomas Small blue cell arising from cerebellum or 4th ventricle, aggressive Mets in CNS and Bm, classic, desmoplastic large cell anaplastic histology, sonic hedgehog pathway Posterior fossa sxs (front of brain) Headache, vomiting, ataxia, nystagmus, mutism, speech disturbances, dysphasia, decreased motor function, emotional lability, cranial nerve palisies, diplopia; 24-100 hours post surgery 100cGy Amount of brain radiation associated with significant late effects Neuroblastoma Malignancy of the parasympathetic nervous system, develops from neural crest cells, found in form of mass in neck, chest, abdomen, often in adrenal gland, silent tumor; most common extra cranial solid tumor in children ICP sxs Morning headaches, lethargy, N/V Supratentorial sxs (back of brain) Heiparesis, seizures, visual changes, intellectual problems (back of brain) Neuroblastoma patho Small round blue cell neoplasm Horner syndrome in NB Unilateral ptosis, myosis, anhydrosis, Neuroblastoma clinical sxs Urine catecholamines, VIMA (vanillymadelic acid) and HVA (homovallnicllic acid) elevated Blueberry muffin sign in NB SubQ nodules appear hardened bluish nodules that can be seen or palpated; often seen with infants with favorable tumors Mibg Scan sensitive to NB, useful in determining metastic disease requires use of potassium iodine to protect thyroid radiation exposure Favorable NB Less than 1yo, hyperdiploid, stage 1, 2, or 4s, primary tumor chest neck/pelvis, high TRKA expression Poor characteristics of NB Increased copies of n-myc, increased ferritin, primary tumor in abdomen, rapid tumor progression Osteosarcoma Associated with p53 tumor suppressor gene Osteosarcoma Production of osteoid substance Genetic associations with osteo Hereditary retinoblastoma, li-faumeni syndrome, Roth mind Thompson Osteo sxs Dull aching pain, increasing at night, averages 3 months before diagnosis, possible soft tissue swelling Distal femur, proximal tibia and humerus Most common sites of osteo (long bone) Lungs Most common relapse site of osteo Surgical reaction in osteo Goal is to completely remove tumor with healthy cell margin of at least 1/2 cm around cancer site Rotationplasty Excision of femur and preservation of lower leg then lower leg turned 180 degrees Osteo surgery options Limb salvage, athrodesis, amputation, rotation plasty Ewing's Undifferentiated, malignant tumor of the bone and soft tissue, radiation sensitive Ewing's sxs Bone pain intermittent 3-9 months, palpable mass, fractures common, primary sites extremity 53% and central 47% Ewing's labs Increased ESR, increased WBC; often mistaken for osteomyelitis Genetic condition with sunburst Rotham thompason with osteo Wilms Tumor of kidney; large rapidly growing vascular abdominal tumors, gelatinous capsule Incidence and etiology of Wilms Beckwith wiseman, denys drash,; 2-3 years older, aniridia Aniridia Absence of iris; in wilms tumor Presentation of wilms Abdominal mass often found by parent bathing patients or picking up child, fever, hematuria, malaise, pain, elevated BP Rhabdomyosarcoma Malignant tumor of the mesenchymal cell origin; often formed skeletal muscle; sMall round blue cell tumor Retinoblastoma Characterized by cells with scant cytoplasm and hyper chronic nuclei of various sizes, arranged in tight bouquet like cluster called flexner winterstiner rosettes leukocoria Cats eye reflex; lack of normal reflex either unilateral or bilateral most common presenting signs for retinoblastoma Sxs of retinoblastoma Strabismus, eaotropia, decreased visual acuity, inflammatory signs Fundoscopic exam Eye exam for retinoblastoma