Ped Oncology - Exam 3 Flashcards
Cancer is the ____ cause of death in children
4th
_____ is the MC malignancy in children. What is the underlying cause? What percent?
ALL
Leukemic blasts replace bone marrow leading to uncontrolled proliferation of immature lymphocytes
More than ____ blasts in the bone marrow aspirate
What is the MC pt for ALL? What age range? What is the peak age?
male white
2-10 years old
peak age is 4
What parent cell does ALL affect? Draw the tree
lymphoid stem cell -> lymphoid blast that then differentiates into B and T lymphocytes
ALL affects the lymphoid blast cell
Intermittent fevers, bone pain, pallor, petechiae, easy bruising, purpura, progressive weakness, dyspnea, infection, fatigue, bone pain
What am I?
What are the s/s related to?
What are 3 common PE findings?
What is a common lab finding?
ALL
Related to decreased bone marrow production of RBCs, WBCs or platelets
enlarged liver and spleen, lymphadenopathy
may have anemia
What lab is the most useful in diagnosis ALL? **What will you see on peripheral smear?
CBC with diff - most useful
lymphoblasts on peripheral smear
What will the CBC show in a pt with ALL? What will the ANC level be? What will the CBC show?
95% have a decrease in at least one cell type
ANC usually <1000, even if WBC count is normal
pancytopenia: all 3 lines are decreased
What am I? What dx?
Lymphoblasts
ALL
_____ is needed for confirmation in ALL. What will it show?
Bone marrow aspirate and biopsy
> 25% blasts in aspirate
Why should you do a lumbar puncture in ALL?
Lumbar puncture to check for CNS involvement and to administer intrathecal chemo
What is the underlying cause of ALL?
translocation of chromosomes 12 and 21
What are the 3 phases of tx for ALL?
phase 1: Remission Induction Phase
phase 2: Intensification Consolidation Phase
phase 3: Continuation Therapy / Maintenance
What is the tx for ALL? What % does this usually cure?
Chemo (ex. vincristine, asparaginase, etc.) with dexamethasone/prednisone and/or intrathecal methotrexate (MTX)
95% remission on bone marrow aspirates
then move on to phase 2: Intrathecal chemo & possibly radiation to kill lymphoblasts in meninges
then phase 3:
Daily, oral chemo, weekly methotrexate, pulses of IV chemo and oral steroids
What are risk factors for ALL? What is the cure rate?
Prenatal and postnatal exposure to x-rays
Down’s Syndrome
overall cure rate is over 90%
______ is the 2nd MC leukemia in children. What does it primarily affect?
AML
Primarily a cancer of bone marrow and lymph nodes
How does Acute Myeloid Leukemia
(AML) compare to ALL?
AML is more aggressive than ALL and can result in death within weeks if not treated
Early flu-like symptoms
Fatigue
Bleeding
Infection
Adenopathy
Skin nodules
Hepatosplenomegaly
What am I?
How do you dx?
AML
CBC with diff and bone marrow aspirate with bx
What will the CBC with diff show in AML? What will the bone marrow aspirate show?
neutropenia (69%)
anemia (44%)
thrombocytopenia (33%)
Again , pancytopenia
30% or more BLASTS with histochemical staining and morphology of leukemic cells
Draw the diagram that shows where the cancer is located for AML on the stem cell tree
What am I? What dx?
Auer rods
AML
What is the tx for AML?
Chemo - systemic and intrathecal - aggressive induction therapy (80-85% remission)
bone marrow transplant
cord blood transplant
What is the expanded tx for AML?
remission induction chemo for 1 month to try and kill as many leukemic cells as possible!!
then phase 2: Stop spread of cancer to brain and spinal cord even if none detected
Intrathecal chemo and sometimes radiation
then phase 3: 1-2 treatments lasting 2 months at a time for approximately 9 months
How long does phase 3: intensification therapy last in AML?
Once in remission - high dose of chemo to kill any remaining cells
1-2 treatments lasting 2 months at a time for approximately 9 months
What are the risk factors for AML? What % of patients will go into remission? What is the long term prognosis?
Neurofibromatosis
Downs
Ionizing radiation therapy
Previous chemo
75-85% remission (compared with 98% ALL)
Still only about 35-50% long-term survival
_______ account for 50% of lymphomas in children. What sex? What age range?
Hodgkin’s Lymphoma
males
before adolescence (10-19) and then again over 50
________ cell in Hodgkin’s Lymphoma undergo malignant transformation for unknown reasons
Germinal center B cells
What are the 3 different forms of Hodgkin’s lymphoma?
childhood form
young adult form
older adult form
When is childhood form of Hodgkin’s lymphoma seen? What increases the risk? What decreases risk?
childhood form: HL
younger than 14
increased risk:
large family size
decreased socioeconomic status
decreased risk:
Early exposure to common infections in childhood decreases risk (think kids who go to daycare)
What age range do you see young adult form of HL? What are the 2 risk factors?
15 - 34 years old
Higher socioeconomic status
earlier birth order (oldest child)
Painless cervical lymphadenopathy
Mediastinal mass
Fatigue, weight loss, anorexia, night sweats and cough
What am I?
What are the “B” symptoms?
Hogdkin’s Lymphoma
Fever (>38 degrees Celsius)
Weight Loss (10% or more)
Drenching night sweats
What will the CBC show in a pt with HL? ESR? _____ approximately 20% of these tumors are associated with it
CBC often normal
Elevated ESR
elevated EBV titers
______ is needed for dx of HL. **What will it show? How would you want to confirm?
bx of node with FNA
**Reed-Sternberg cell on biopsy
confirm with bone marrow aspirate and bx and LP to see if the cancer has reached the bone and spinal fluid
What am I? What dx? What is an important thing to note?
Reed-Sternberg cell
Hodgkin’s Lymphoma
abnormal lymphocytes that may contain more than 1 nucleus
What are the suspicious lymph node bx criteria?
Lack of infectious cause
Lymph node > 2 cm
Supraclavicular node
Abnormal CXR
Increasing node size after 2 weeks of antibiotic treatment, OR failing to decrease in size in 4-6 weeks
What is the tx for HL? What is disease free survival percentage? What does the prognosis correlate with?
chemotherapy!!
Disease free survival at 90-95% = Cure
Prognosis correlates with “B symptoms”
What is the underlying cause of non-Hodgkin’s lymphoma? It arises from ______
Immature lymphocytes grow out of control and fail to mature and accumulate in lymph tissue (nodes, spleen, thymus)
arises from lymphoid cells
_____ is the 3rd MC cancer in kiddos. What sex? What race?
Non-Hodgkin’s lymphoma
male
white> black
What are the 3 organ systems involved in NHL?
spleen
thymus
lymph
Cough, dyspnea, orthopnea, facial edema, lymphadenopathy, mediastinal mass, pleural effusion, abdominal pain or distention
What am I?
Where are the 2 MC sites for these tumors to spread?
NHL
abdomen #1
chest #2
What are the 3 types of NHL?
Burkitt Lymphoma (Small noncleaved cell lymphoma
Lymphoblastic Lymphoma
Large Cell Lymphoma
What is the MC pt in Burkitt Lymphoma?
boys 5-10 years old
Burkitt’s lymphoma originates from _____ phenotype. How common is it when compared to other types of NHL?
Originates from a B-cell phenotype
40-50% of cases of NHL
Burkitt Lymphoma tend to present with ______ tumor. What is the genetic cause?
Intra-abdominal tumor that grows very fast and may block bowels
Chromosome 8
What is the MC pt with lymphoblastic NHL? How common is it? What cell does it originate from?
teen boys
25-30% of cases of NHL
Originate in immature T-cells -> T-cell surface markers
What is the genetic cause of lymphoblastic NHL? How long is therapy for?
Translocation of Chromosome 14q11 and 7 with deletion of chromosome 1
15-18 month therapy like ALL
How can you tell lymphoblastic lymphoma NHL apart from ALL?
Indistinguishable from ALL
If >25% blasts in bone marrow - ALL not NHL
cough, dyspnea and orthopnea
Mediastinal mass 50-70%, Lymphadenopathy elevating diaphragm (50-80%)
What am I?
How common is it?
Lymphoblastic Lymphoma NHL
25-30% of cases of NHL
What cell lines are affected in large cell lymphoma? What tumor? How common is it?
Abdominal tumor most common
B and T-cell or Histiocytes
< 15% of cases
How do you dx NHL? What lab reflects tumor burden and serves as a marker for disease activity?
Bone marrow aspirate and biopsy
LDH
What are the risk factors for NHL? How would you describe the tumor?
organ transplants
EBV
HIV
radiation exposure
tumor is typically aggressive but responds well to treatment
What is the prognosis in NHL?
5 year survival: those who live at least 5 years after diagnosis and free of disease are likely cured (90%)
What are the triad of brain/spinal tumors? How common are they?
morning headache, vomiting and papilledema (30%)
25-30% of all childhood cancers
What is the MC solid tumor of childhood? ____ and _____ are glial cell tumors
brain/spinal tumors
Glial Cell - Astrocytomas and Ependymomas
Non-glial cell tumors include ______ and _____. How do you dx?
Medulloblastomas and other primitive tumors
MRI w/ and w/o contrast
What is the brain location breakdown of brain tumors?
Where are astrocytomas commonly found?
in the cerrebellum
closer to the front
_______ the MC brain tumor of childhood and are found in the _______. How do you dx?
astrocytomas
cerebellum
MRI mainstay of diagnosis and confirmed via biopsy, CSF cytology
What are the different grades of astrocytomas? What are the major differences?
WHO grade I- IV
WHO grade I-II: slow growing, least serious which make up 80% of astrocytomas
WHO grades III-IV: fast growing and malignant
What is the MC types of astrocytoma? What is the tx?
WHO Grade I (Juvenile pilocytic) most common - slow growing and cystic requiring surgical removal only
What is the tx for an astrocytoma? What is the 5-8 year survival rate?
Treatment - steroids and anticonvulsants prior to surgery followed by high dose systemic chemo
5-8 year survival: 60-90%
Where are brain stem gliomas found? What is the tx? What age group?
Middle of brainstem
Challenging to treat - majority no removal- NO SX!!! usually just chemo/radiation
Exclusively in school age children
_______ are ______ that are found in the lining of the ventricles or spinal cord near cerebellum. Why are they bad?
Ependymoma
gliomas
blocks CSF flow
Are ependymoma fast or slow growing? What age?
Slow growing
5 year old peak age
What type of gliomas are common to see in pts that also have neurofibromatosis?
Optic Nerve Gliomas
______ is the MC malignant brain tumor. What age range? What sex? Where do they like to metastasize?
Medulloblastomas (Non-Glial Cell) in the cerebellum
5-10 years old
Boys > Girls
spinal cord
What is a neuroblastoma? What age? Where are they primarily found?
Tumor of nerve tissue that is extremely malignant
usually under age of 5 (90%)
Primarily found in the adrenal gland
______ are the MC extracranial solid cancer in childhood and most common cancer in infancy. Where does it arise from? What sex?
Neuroblastoma
Arises from neural crest tissue of synthetic ganglia or adrenal medulla
Boys > Girls
Where are the 2 MC locations to find a neuroblastoma? Will it cross over the midline?
1 adrenal gland
neuroblastomas WILL cross over midline!!
Abdominal mass - firm, fixed, immobile, and irregularly shaped extending beyond midline (65%), adenopathy and HSM
Bone pain, abdominal pain, anorexia, weight loss, fatigue and fever - constitutional symptoms
What am I?
What age?
**What is the highlighted finding?
Neuroblastoma
< 2 years old at diagnosis most common
Abdominal mass - firm, fixed, immobile, and irregularly shaped extending beyond midline
What lab tests should you order in neuroblastoma?
CBC for anemia (60%)
**Urinary Catecholamines (Urine VMA/HVA test) - tumor secrets this and it will be increased in 90% of patients at diagnosis, thrombocytopenia
What is the tx for neuroblastoma? What is an improved prognosis? How likely it is to metastasize?
Surgical removal, chemo and radiation
<1 year old and small tumor - improved prognosis and possibly only surgical removal
High rate of metastasis and non-responsiveness to treatment/ high recurrence
What is another name for a nephroblastoma?
“Wilms Tumor” on one or both kidneys
How will a nephroblastoma present?
Asymptomatic abdominal mass or swelling (>80%) that does NOT cross midline but may be mobile or displaced
_____ is the 2nd MC abdominal tumor. Where does it arise from? What race? How are they usually found?
Nephroblastoma
Arises from kidney
African Americans
Presents as an asymptomatic abdominal mass found by parents or increased size of abdomen
What age range is associated with a nephroblastoma? What imaging should you order?
Uncommon over age of 5 (usually 2-5 y/o)
U/S and then CT abdomen
Abdominal pain, fever, N/V, hematuria, elevated BP - but these constitutional symptoms not common, usually asymptomatic
Abnormally large abdomen
What am I?
Where is the MC site of metastasis?
What is the 5 year survival?
Nephroblastoma
lungs
5 year survival around 90%
Where in the bone are osteosarcomas more likely to be found? Ewing’s sarcoma?
Osteosarcoma: metaphysis
Ewing’s sarcoma: diaphysis
______ are the MC bone cancer in children and adolescents. What age range? What sex?
Osteosarcoma
10 -19 years old most common
males> females
Why does osteosarcoma occur?
Cancer affecting osteoblasts
2 suppressor genes (p53 and Rb mutations)
What are some PE findings in osteosarcoma?
often found in adolescents around the time of a major growth spurt due to rapid bone growth
kiddos are sometimes taller than peers due to greater increase in length and size of bones
What bones are osteosarcomas more likely to involve? Where along the bone?
Long, tubular bones primarily affected (distal femur - 40%, tibia, humerus)
metaphysis
lump, limp and wakes at night with pain
What am I?
Where are metastasis more likely to occur?
What labs?
Osteosarcoma
pulmonary metastasis
CBC, CMP with Alk phos and LDH
What is the tx for osteosarcoma? Has a strong genetic link with ______
chemo both PO and IV and limb salvage
retinoblastoma
What is the prognosis for osteosarcoma? What is the 5 year survival rate?
15 - 20% already have mets at time of diagnosis and >70% receiving surgery alone develop pulmonary mets within 6 months after surgery
55-80% survival at 5 year follow-up
What are the risk factors for osteosarcoma?
age, male, tall stature, possibly radiation exposure
What are some xray findings associated with osteosarcoma?
sun burst appearance
Codman’s triangle
What is the underlying cause of Ewing’s sarcoma? What does it due to future risks?
Genetic change in a primitive cell after birth
Increased risk for other cancers in future
_____ is the 2nd MC bone cancer in children and adolescents. Where does it occur in the bone? What sex?
Ewing’s Sarcoma
Diaphysis of long bones ( middle - long part )
males> females
What is Ewing’s sarcoma commonly misdiagnosed as?
Misdiagnosed as “growing pains”
Present with soreness at tumor site and swelling with warmth to touch
Worse with exercise or at night, tender lump or limp
What am I?
What lab?
What is the tx?
Ewing’s sarcoma
may have elevated LDH
Chemo (IV) before surgery for 6-9 months, then removal of tumor
Ewing’s sarcoma has a ___ long term disease free survival if no metastasis
50-70%
What is a retinoblastoma? What does it arise from?
Tumor of the retina - necrotic in center with areas of hemorrhage
Arises from embryonic retinal cells - mutation in RB1 gene in long arm of chromosome 13
How is a retinoblastoma commonly found? What is the underlying cause if bilateral?
Most picked up by the parents or caretakers , Cat’s eye
Bilateral eyes - hereditary
____ is known as a “white pupillary reflex” and is a common sign for retinoblastoma
Leukocoria
How do you dx a retinoblastoma?
Diagnosis by CT scan of orbits, followed by MRI, bone scan, bone marrow and LP
Examination of eye under anesthesia with dilated pupils
What is the tx for a retinoblastoma?
External beam radiation is mainstay of tx
if that does NOT work then removal of eye and 90% need no further treatment
What is the 5 year survival rate of a retinoblastoma?
5 year survival rate is 90%
