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Peds Exam 3
Oncology/Childhood Cancers pt 1
| Question | Answer |
|---|---|
| oncology and childhood cancers are the | leading cause of death from disease |
| oncology and childhood cancers affect | the entire family |
| incidence of oncology and childhood cancers | 1 in 285 children higher incidence in <4 and 15-18 years old slightly higher in males than females |
| Specifics regarding Cancers of Childhood | HPV can lead to cancer unregulated cell division radiation is a known cause |
| Childhood Leukemia affects | WBCs |
| Childhood Leukemia involves the | Unrestricted proliferation of immature WBCs (blasts) |
| leukemia infiltrates | organs and tissues |
| leukemia depresses | production of normal blood cells in marrow since it crowds out RBC and platelets |
| major presenting symptoms of leukemia are secondary to | marrow suppression (infection, anemia, bleeding) tissue infiltration (pain, organomegaly) |
| why does organomegaly happen in leukemia | blasts spill out of bone marrow and into tissues/organs |
| Classification Childhood Leukemia | classified by how quickly onset is and which cell line is affected |
| progression of leukemia | Acute- immature cells (blasts)-multiply rapidly Chronic- partly mature, replicate/ accumulate more slowly |
| cell line of leukemia | Lymphocytic (ALL, CLL) Myelocytic (AML, CML) |
| ALL | most common acute lymphocytic |
| CLL | chronic lymphocytic |
| AML | acute myelocytic |
| CML | chronic myelocytic |
| what does leukemia affect | myeloid stem cell and lymphoid stem cell |
| blast cell is | immature blood cell |
| Acute Lymphoblastic Leukemia (ALL) is most common | childhood malignancy |
| Acute Lymphoblastic Leukemia (ALL) peaks | in early childhood (2 - 5 years) but has a 90-95% success rate |
| ALL is classified according to | (immature) lymphocyte cell line |
| which lymphocyte cell lines do ALL affect | B-cell -most common 85% cases T-cell |
| T-cell ALL aka | T: terrible more severe, more aggressive Tx, and not as favorable Dx |
| CMs of ALL | Anemia Neutropenia Thrombocytopenia Leukemic cell infiltration T-cell ALL- enlarged thymus |
| Anemia due to ALL | decreased RBCs so will be fatigued and pale |
| Neutropenia due to ALL | abnormal/immature WBC which causes: Fevers (no known cause) Repeated infections (bc immunosuppressed) |
| Thrombocytopenia due to ALL | decreased platelets that cause: Bleeding, bruising easily/without cause, petechiae (bleeding under skin) |
| Leukemic cell infiltration due to ALL | Bone, joint pain, limping Enlarged liver, spleen, lymph nodes- abdominal pain |
| T-cell ALL causes an enlarged thymus which can lead to | Respiratory distress |
| Diagnosis of ALL: suspected | aka symptoms are recognizable History, physical, peripheral blood smear- blasts and low blood counts (results come quickly) Elevated WBC – 50% |
| what is prognosis of ALL based on | if WBC are increased during diagnosis |
| Definitive Diagnosis of ALL | bone marrow aspirate/biopsy- infiltrate blasts Posterior superior iliac crest (hip) - minor discomfort post op |
| additional tests for ALL | CXR to see if any masses Lumbar puncture (LP) to see CNS involvement |
| Prognostic indicators of ALL | Age at diagnosis WBC at diagnosis CNS involvement Sex Cytogenetics/genomic alteration Treatment response |
| Sex in ALL | girls better prognosis in boys, treatment lasts 6 months longer |
| Cytogenetics/genomic alteration in ALL | survival better in B-cell |
| Age at diagnosis of ALL | Favorable > 1 year or < 10 years Unfavorable- infants |
| WBC at diagnosis of ALL | Favorable < 50,000/mm3 – B cell ; < 100,000- T-cell |
| Treatment response of ALL | favorable rapid response at end of induction |
| Treatment of ALL could last | 3-4 years but have great prognosis |
| Treatment of ALL | Combination chemotherapy, 3/4 phases Hematopoietic stem cell transplantation (HSCT) reserved for refractory/relapsed disease |
| Combination chemotherapy, 3/4 phases | Remissive Induction Consolidation/ intensification Maintenance |
| Remissive Induction | very intense, hospitalized during this phase Goal is to achieve remission (less the 5% blasts in bone marrow) Steroids (to prevent tumor lysis syndrome) and chemo |
| Consolidation/ intensification | looking for any remaining cancer cells especially in CNS to eradicate residual leukemic cells, particularly in CNS intrathecal chemotherapy - into CSF via LP cranial radiation for highest risk |
| intrathecal chemotherapy - into CSF via LP because it | overcomes blood-brain barrier |
| cranial radiation for highest risk during consolidation/ intensification | kills any cells in CNS, radiation to developing brain causes issues so ideally do this treatment after 3 years but definitely after 1 year! |
| Consolidation/ intensification and Maintenance can be done | outpatient |
| maintenance phase | preserve remission & reduce risk of relapse |
| Hematopoietic stem cell transplantation (HSCT) is done if | cancer is not going away |
| anything <85% adherence, | increased risk for relapse generally same regimen in different hospitals |
| Lymphoma is the | malignancy of lymphoid tissues overgrowth of lymphocytes |
| Lymphomas | Neoplastic disorders arising from lymph tissue |
| classification of lymphomas | Hodgkin Disease (HD) or lymphoma Non-Hodgkin Lymphomas (NHL) |
| lymphomas affect | B lymphocytes |
| classification of lymphomas | A- Asymptomatic, but often enlarged lymph nodes B –presence of fever, night sweats (so will have to change sheets), weight loss of 10% or more |
| complications of lymphomas | Liver failure Can develop secondary cancer- acute nonlymphocytic leukemia (NHL) |
| survival rate in lymphomas | > 90% 5-10 year survival |
| Hodgkin Lymphoma starts in | one enlarged lymph node but progresses to lymph node since itd touching others, it spreads |
| Hodgkin Lymphoma is less aggressive unless | B symptoms, has slower onset |
| Hodgkin Lymphoma primarily involves | superficial lymph nodes |
| Hodgkin Lymphoma affect on superficial lymph nodes | Supraclavicular or cervical Enlarged, non-tender, painless, “rubbery” and movable Initially single node, then cluster |
| Hodgkin Lymphoma causes | Unexplained low grade or intermittent fever Anorexia, nausea, weight loss Night sweats and pruritus Respiratory distress if mediastinal mass |
| incidence of Hodgkin Lymphoma | Rare before age 5, increased after age 15 and into young adulthood Link to Epstein Barr virus but still don't understand why |
| Diagnosis of Hodgkin Lymphoma | H/P, CXR, CT, MRI, PET Scan Biopsy of lymph node |
| Biopsy of lymph node to diagnosis Hodgkin Lymphoma | Presence of Hodgkin and Reed-Sternberg cells is diagnostic Staged (I-IV) |
| Reed-Sternberg cells | large abnormal cell, binuclear |
| Hodgkin Lymphoma is staged based on | A or B and what region of lymph nodes are affected |
| treatment of Hodgkin Lymphoma | Chemotherapy Radiation- possibly HSCT |
| Non-Hodgkin Lymphomas (NHL) affects which lymphocytes | B and T lymphocytes |
| NHL is more 1.5x more prevalent than | Hodgkin's in children very aggressive in kids, not in adults |
| NHL onset | Rapid onset- days to weeks before symptoms |
| NHL affects | deep lymph nodes-(neck and chest) spreads in bloodstream |
| NHL cells | undifferentiated, infiltration diffuse |
| NHL is a "bulky disease" | mediastinal, abdominal, marrow and rapid cell reproduction can precipitate life-threatening emergency |
| NHL causes | obstruction metabolic (tumor lysis syndrome) - treated by Allopurinol and hydration |
| NHL compared to HL symptoms | Similar symptoms of HL but metastasis to bone marrow and CNS produce leukemia like symptoms |
| NHL is typically | widespread at diagnosis, found outside of lymph node |
| Treatment of NHL | Multi-agent chemo CNS prophylaxis Radiation limited Autologous (own blood cells) bone marrow transplant |
| Multi-agent chemo to induce | remission, then maintenance for 2 years |
| CNS prophylaxis because | can easily spread to CNS |
| brain tumors start in | brain |
| presentation of brain tumors depends on | location & histology of tumor, and tumor grade (how aggressive) |
| most common childhood brain tumors | Astrocytoma, Brainstem glioma, Medulloblastoma and Ependymoma |
| treatability of childhood brain tumors is a function on | both grade (rate of growth) and location (surgical respectability) |
| brain tumors are the | 2nd leading cancer in peds |
| Brain tumor CMs are directly related to | location/ size of tumor and age of child |
| classic symptoms of brain tumors | Headache, especially upon awakening |
| CMs of brain tumors | Vomiting not related to feeding Increased intracranial pressure- related to obstruction and hydrocephalus Balance issues, visual disturbances, seizures, behavioral changes, hemiparesis, growth failure, diabetes, abnorm reflexes, pos Babinski (> 1 yr) |
| why is there increased ICP with brain tumors | tumor obstructing CSF |
| visual disturbances due to brain tumor | papilledema - swell of optic discs, more lower grade |
| brain tumors have | Sudden onset -more aggressive tumors |
| diagnosis of brain tumors | subjectively on presenting clinical signs objectively on neurologic tests and histologic diagnosis via surgery (biopsy) |
| primary treatment of brain tumors | surgery |
| surgery for brain tumors | Goal- most extensive resection, preserving neurological function Curative only in low-grade tumors with clear demarcation |
| clean margins means | all around the circumference of tumor is healthy tissue |
| other treatments for brain tumors | radiation and chemotherapy |
| radiation | Standard treatment children >3 years old Irradiating developing brain is problematic especially under 1 year old |
| Irradiating developing brain is problematic especially under 1 year old | cognitive impairment growth retardation sensory impairment |
| chemotherapy for brain tumors | Usually part of multi-modal therapy |