Oncology Flashcards
4th leading cause of death in children
CA
MC malignancy in children (nearly 1/3 of all pediatric cancers)
Acute Lymphoblastic Leukemia
(ALL)
- Leukemic blasts replace bone marrow
- Uncontrolled proliferation of immature lymphocytes
- >25% blasts in bone marrow aspirate
- Unknown cause - but may have a genetic link
- Peak age: 4 years old (usually 2 - 10 yo)
dx?
Acute Lymphoblastic Leukemia
(ALL)
A blood stem cell goes through several steps to become a ? (3)
RBC
WBC
platelet
Where does CA start in regard to stem cell development?
- after differentiating from a common lymphoid progenitor
- before becoming a NK cell and smal lymphocyte
ALL develops from what type of stem cell?
lymphoid blast
s/s of ALL
- dec bone marrow production of RBCs, WBCs or platelets
- fevers, bone pain, pallor, petechiae (and easy bruising), purpura, progressive weakness, dyspnea, infection, fatigue
- Enlarged liver and spleen, Anemia
- LAD
w/u for ALL?
-
CBC w/ diff
- 95% have dec in at least one cell type - neutropenia, thrombocytopenia or anemia
- ANC < 1k, even if WBC normal - peripheral smear
- BMP, Uric Acid, LDH, PT/PTT
- DX: BM aspirate
- LP
- CXR
- Lymphoblasts on peripheral smear
- Pancytopenia (all 3 cell lines are decreased)
likely dx?
ALL
ALL - Abnormal white cells reveal hyperdiploidy and translocation of chromosomes ___ and ____
12 and 21
how to perform BM aspirate and bx?
- numbed
- insert Jamshidi needle (a long, hollow needle) hip bone
- Samples of blood, bone, and bone marrow are removed for examination under a microscope
mgmt for ALL
- phase 1: Remission Induction - chemo + dex/pred +/- intrathecal MTX
- Phase 2: Intensification Consolidation Phase - Intrathecal chemo +/- radiation
- Phase 3: Continuation Therapy / Maintenance - Daily, oral chemo, wkly MTX, pulses of IV chemo and oral steroids
Treatment for 15 - 18 months, overall cure rate 90%
goal remission % during phase 1 for ALL?
95% remission on bone marrow aspirates
RF for ALL
- XR
- Downs syndrome
- 2nd MC leukemia in children
- Untreated can result in death within weeks
- Primarily a cancer of bone marrow and lymph nodes
Acute Myeloid Leukemia
(AML)
Also called Acute Myelogenous Leukemia
s/s of AML
- Early flu-like sx
- Fatigue
- Bleeding
- Infection
- Adenopathy
- Skin nodules
- HSM
w/u for AML
- CBC w/ diff
- BM aspirate
- LP, CXR
- CBC reveals neutropenia, anemia, and thrombcytopenia
- Bone marrow aspirate shows 30% blasts with histochemical staining and morphology of leukemic cells
- Peripehral smear shows auer rods
dx?
AML
AML comes from what stem cell?
myeloblast - either myeloid stem cell or myeloid blast
general mgmt approach of AML
- Less responsive, more intense
- Chemo - systemic and intrathecal - aggressive induction therapy (80-85% remission)
- Radiation - sometimes
- BM transplant
- Cord blood transplant
tx phases of AML
- Phase 1: Remission Induction Chemo - 1 month; Kill as many leukemic cells ASAP = cancer goes into remission
- Phase 2: Consolidation Preventative - Stop spread of cancer to brain and spinal cord; Intrathecal chemo +/- radiation
- Phase 3: Intensification - high dose of chemo; 1-2 tx lasting 2 mo x 9 mo
RF for AML
- Ionizing radiation therapy
- Previous chemo
- Syndromes - Neurofibromatosis, Downs
disposition and prognosis of AML
- Can reoccur
- Remission when WBC in normal range
- 75-85% remission
- 35-50% long-term survival
- Account for 50% of lymphomas in children
- peak before adolescence and again >50 y/o
- Germinal center B cells undergo malignant transformation for unknown reasons
Hodgkin’s Lymphoma
what are the 3 different forms of Hodgkin’s?
- Childhood form - < 14 y, inc risk with large family size, dec socioeconomic status
- Young Adult form - 15 - 34 y, Higher socioeconomic status and earlier birth order (oldest child)
- Older Adult form
what decreases the risk of childhood form Hodgkin’s?
Early exposure to common infections in childhood
- Painless cervical LAD (70-80%) or supraclavicular (25%)
- Mediastinal mass (50-75%) and asx
- Fatigue, wt loss, anorexia, night sweats and cough, fever
dx?
HL
B sx: F, wt loss, night sweats
w/u for HL?
- CBC
- ESR - elevated
- EBV titers
- LN bx
- BM aspirate; LP
- CXR, CT; bone scan; PET
Reed-sternberg cell is associated with what dx?
HL
suspected HL, Early bx of enlarged LN if:
- Lack of infectious cause
- Lymph node > 2 cm
- Supraclavicular node
- Abnormal CXR
- Increasing node size >2 wks of abx, or failing to dec in size in 4-6 weeks
tx and prognosis of HL
- Chemotherapy
- Disease free survival at 90-95% = Cure
- Prognosis correlates with “B sx”
- Immature lymphocytes grow out of control and fail to mature and accumulate in lymph tissue (nodes, spleen, thymus)
- Arise from lymphoid cells
Non-Hodgkin’s Lymphoma (NHL)
3rd MC cancer in children?
Non-Hodgkin’s Lymphoma (NHL)
s/s of NHL
Cough, dyspnea, orthopnea, facial edema, LAD, mediastinal mass, pleural effusion, abdominal pain or distention
MC site of tumor for NHL
- abdomen
- chest
- Boys 5 - 10 y/o
- Originates from a B-cell phenotype
- MC cases of NHL
- Intra-abdominal tumor (MC), Jaw, or bone marrow
- Chromosome 8
- abdominal pain, distention, RLQ mass
- Fastest growing tumor - can block bowels
which type of NHL
Burkitt Lymphoma (Small noncleaved cell lymphoma)
- Teen boys
- Originate in immature T-cells (T-cell surface markers)
- 25-30% of cases of NHL
- Mediastinal mass 50-70%, LAD elevating diaphragm (MC)
- MC airway compression sx: cough, dyspnea and orthopnea
- Indistinguishable from ALL
- Translocation of Chromosome 14q11 and 7 with deletion of chromosome 1
which type of NHL? mgmt?
- Lymphoblastic Lymphoma
- 15-18 month therapy like ALL
how to differentiate lymphoblastic lymphoma vs ALL?
If >25% blasts in bone marrow - ALL not NHL
- B & T-cell or Histiocytes
- < 15% of cases
- MC Abdominal tumor
which type of NHL
Large Cell Lymphoma
w/u for NHL
- Bx + Histology with tissue removal
- LDH - reflects tumor burden, marker for disease activity
- CXR, CT chest, U/S, Bone scan, ECHO, EKG and PFTs
- BM aspirate and bx
RF for NHL
- organ transplants
- EBV
- HIV
- radiation exposure
mgmt and prognosis for NHL
- Chemo +/- Radiation
- 5 year survival - after 5 years after dx of NHL-free = cured (90%)
classic triad of a brain/spinal tumor?
morning HA, vomiting and papilledema (30%)
s/s of brain/spinal tumor seen in older vs younger child?
- Older children - personality changes, school issues (failure)
- Younger children - Irritability, FTT, Dev Delay
- 25-30% of all childhood cancers
- MC solid tumors of childhood
Brain & Spinal Tumors
what tumors are seen with gial cells?
- Astrocytomas
- Ependymomas
what tumors arise from nonglial cells?
Medulloblastomas and other primitive tumors
w/u for brain/spinal tumors?
MRI w/ & w/o contrast
what % of tumors occur in the tentorium and posterior fossa?
50/50
- MC brain tumor of childhood (about 50%)
- In the cerebellum
Astrocytoma
Grading for Astrocytoma?
- WHO Grade I (Juvenile pilocytic) MC - slow growing and cystic; surgical removal only
- WHO Grades I-II - slow growing, least serious; 80% of astrocytomas
- WHO Grades III-IV - fast growing and malignant
w/u and mgmt for Gliomas (Glial Cell Tumors)
- MRI and confirmed via bx, CSF cytology
- steroids + anticonvulsants before surgery; high dose systemic chemo after
- 5-8 year survival 60-90%
- Middle of brainstem
- Challenging to treat - majority no removal
- Exclusively in school age children
which type of glioma?
brain stem
- Lining of ventricles or spinal cord near cerebellum
- Blocks CSF flow
- Slow growing
- 5 year old peak age
which type of glioma?
Ependymoma
Optic Nerve Gliomas Common in what condition?
Neurofibromatosis
- MC malignant brain tumor
- 15% of children
- Cerebellum
- 5-10 y; Boys > Girls
- Metastasis to spinal cord
Medulloblastomas (Non-Glial Cell)
Tumor of nerve tissue
Extremely malignant neoplasm
MC extracranial solid cancer in childhood and MC cancer in infancy (< 5y/o (90%))
Neuroblastoma
Neuroblastoma is primarily found where?
adrenal gland
T/F: Neuroblastoma can be inherited
T
s/s of neuroblastoma
- constitutional sx - bone pain, abd pain, anorexia, wt loss, fatigue and F
-
Abdominal mass - firm, fixed, immobile, and irregularly shaped extending beyond midline (65%), adenopathy and HSM
< 2 years old MC
w/u for neuroblastoma and findings?
- CBC - anemic, thrombocytopenia
- Urine VMA/HVA test - Urinary Catecholamines
- Imaging: XR, CT, skeletal survey, bone scan
- Tissue bx for staging and BM aspirates and bx
mgmt and prognosis for neuroblastoma
- Surgical removal, chemo and radiation
- < 1 y/o + small tumor - improved prognosis and possibly only surgical removal
- High rate of metastasis and non-responsiveness to tx/high recurrence
Aka “Wilms Tumor” – one or both kidneys
Nephroblastoma
asx abdominal mass or swelling (>80%) - does not cross midline but may be mobile or displaced
dx?
w/u?
- Nephroblastoma
- U/S, CT or MRI
2nd MC abdominal tumor?
Nephroblastoma
Nephroblastoma affects what pt demographic MC?
- African Americans
- MC sporadic, genetic or syndromic link possible
- 2-5 y/o
how to stage for nephroblastoma?
Nephrectomy, Chemo or Radiation, Clinical Trials
MC of met for nephroblastoma?
lungs
difference between Osteosarcoma vs Ewing’s sarcoma?
- Osteosarcoma - metaphysis
- Ewing’s sarcoma - diaphysis
Most common bone cancer in children and adolescents (60% of cases)
Osteosarcoma
MC pt demographics of Osteosarcoma
- Adolescents and young adults - 10 -19 years old MC
- Males > Females
Osteosarcoma affects what cells and genes?
- osteoblasts
- 2 suppressor genes (p53 and Rb mutations)
- Adolescent growth spurt, rapid bone growth
- Taller than peers - greater increase in length and size of bones
- Metaphysis of bones
- MC Long, tubular bones (distal femur - 40%, tibia, humerus)
- lump, limp and wakes at night with pain
dx?
w/u?
- Osteosarcoma
- XR, CT/MRI, tissue sample, chest CT, bone scan
- CBC, CMP with Alk phos and LDH for prognostic purposes
MC met site for osteoscaroma?
lung
mgmt and prognosis of osteosarcoma?
- Chemo (PO + IV), Limb salvage (80% of pts)
- Prognosis: bad; may have mets at time of dx, >70% receiving surgery alone develop pulmonary mets < 6 mo after
- 55-80% survival at 5-year f/u (75% local lesion, 30% mets)
RF for osteosarcoma?
- age, male, tall stature, possibly radiation exposure
- Strong genetic link with retinoblastoma
- Genetic change in a primitive cell after birth
- inc risk for other cancers in future
- 2nd MC bone cancer in children and adolescents
- Diaphysis of long bones (middle - long part)
- Misdiagnosed as “growing pains”
Ewing’s Sarcoma
s/s of Ewing’s sarcoma?
w/u?
- soreness at tumor site and swelling with warmth to touch
- Worse with exercise or at night, tender lump or limp
- XR, CT, MRI, bone scan and tumor biopsy; may have elevated LDH level
mgmt for Ewing’s sarcoma
- Chemo (IV) before surgery x 6-9 mo, then removal of tumor
- PT and Rehab - 6-9 months
- 50-70% long term disease free survival if no metastasis
Tumor of the retina - necrotic in center with areas of hemorrhage
Retinoblastoma
pathophys of Retinoblastoma
Arises from embryonic retinal cells - mutation in RB1 gene in long arm of chromosome 13
what makes retinoblastoma more likely to be hereditary?
bilaterally
s/s of retinoblastoma
- Leukocoria - “white pupillary reflex” is a common sign (cat’s eye appearance) - 60%
- Strabismus, painful, red eye w/ glaucoma , proptosis - less common
- Asymmetry of eyes in photos
w/u and mgmt for retinoblastoma?
- CT scan, then MRI, bone scan, bone marrow and LP; Exam eye under anesthesia with dilated pupils
- External beam radiation; Removal of eye
- 5 year survival rate is 90%
