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Peds Exam 3

Oncology/Childhood Cancers pt 1

QuestionAnswer
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
Created by: leh195
 

 



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