Pediatric Oncology 2 Flashcards
Acuke Lymphoblastic Leukemia
Epidemiology:
Presentation:
Tx:
Epidemiology: most common, peak at 2-5 yrs, caucasian males, rogue stem cell, can find anywhere. Bone marrow infiltration,
Presentation: anemia, dec platelets, neutropenia bone pain, Lymphadenopathy, hepatosplenomegaly, orthopnea, Fever of malignancy
Dx: CBC (inc wbc, lymphoblasts on smear, most have 2 cytopenias) these could be other things as well.
association with down syndrome, Risk factor is prebirth radiation
Tx: CHemo for 5-6 cycles
THen maintenance.
Goal is to inc survival. follow for 10 years
Outcomes of tx: 95% attain remission, Late effects on bone neuro development obesity,
Precursor B- ALL
85% of ALL
Present: Marrow involve
Precursor T All
Male, Adolescents, 15% all,
High WBC really high,
Present: Mediastinal mass, high wbc, lymph node involvement, liver spleen
What is NCI ROME?
it is a way of characterizing ALL pnts when they walk in the door based on age and WBC count.
Now we add in Immunophenotype after 4 weeks, then response to therapy.
Acute Myeloid Leukemias
Epidemiology: Not as common as ALL, Prognostic factors are age,race, cytogenetics,
Presentation:
Tx: cytarabine, doxorubicin
50% recurrence, cardiac toxicity
CNS Malignancies
Astrocytomas: low grade gliomas, most common, occur in optic pathway, grade 1.
Signs: inc pressure iCP,
What are clinical features of
Posterior Fossa and brainstem tumors?
Ataxia, tremor, dysarthria, stiff neck, head tilt, papilledema, swallowing prob
What are clinical features of
Hemispheric tumors?
Hemiparesis, aphasia, seizures
Brain tumor workup
CT, MRI with and without contrast,
surgery is best tx, chemo adjuvant - not curable, radiation
What is the epidemiology of common tumors?
astrocytomas:
Li-Fraumeni Syndrome:
astro: assoc with NF-1
Li-Fraumeni: germline mut in p53.
Radiation to head/neck
What is the prognosis of tumors? Astrocytoma? Medulloblastoma? Ependymoma? Brain Stem Gliomas?
Depends on sx removal and aggressiveness of tumor
astro: Low grade: 50-80% survival
Medullo: 70-80% 5 yr
Ependymoma: Resecion: 50-75%, Less resection less than 30%
Brain stem glioma: less than 9 mo
Wilms tumor
2nd most common abdominal tumor WT1 mutation Renal tumors, african, 2-3 yr old. assoc w/: hemihypertrophy, aniridia, Denys-Drash syndrome (GU anomalies), Bekwith-Weidemann syndrome PE: abdominal palpable mass, do it carefully, may rupture and seed Hx: HTN, hematuria, fever, anemia, some with hypotension from blood loss Staging: 1-V - I is not bad, resection - III tumor spill at sx - IV mets to lung - V bilateral renal involvement Prognosis: excellent
Retinoblastoma
absence of red reflex, less than 3, 1% of all tumors
aggressive radiation, chemo
good survival
Neuroblastoma
Unique and fast progressing
occur in any neural crest tissue
Signs: of metatstatic dz, irritable, wt loss, bone pain, fever; proptosis, bone lesions, racoon eyes,
Presentation: large abdom mass crosses midline, weakness,
Most common extracranial solid tumor. average age 18 mo. n-myc amplified,
Staging: 1-4. 4S is infantile that may regress on its own.
- evaluate clinically and imaging, bone marrow, node excision
Prognosis: based on stage at presentation
What are soft tissue sarcomas?
Rhabdomyosarcoma: most under 10. Head/neck (proptosis, cranial nerve pasly, hearing loss), GU (bladder or prostate, hematuria, grape like tumor coming out of vagina, extremities, trunk, retroperitoneal
5% of child malig
Stage 1-4.
Ewing sarcoma: 11-15 peak, male, swelling, sports related injury sx, chemo, radiation survival dep on stage MRI are way better than x ray diaphesis of long bones, and flat bones
Osteosarcoma
Male adolescent injury
tx same as ewing except radiation. Survivial dep on statge
Metaphysis of long bones
