Lecture 22: Pediatric Oncology Flashcards
The 4th leading cause of death in children is…
Cancer
:(
Overall, the MC malignancy seen in children, comprising 1/3 of all pediatric cancers, is….
ALL (Acute lymphoblastic leukemia)
ALL is diagnosed by having greater than ()% blasts in the bone marrow aspirate.
25% lymphoblasts or more!
You have a 4 yo white boy who has intermittent fevers, bone pain, and bruises easily. On physical exam, you notice hepatosplenomegaly. His CBC shows anemia with an ANC < 1000. Lymphoblasts are seen on his peripheral smear. You suspect what malignancy?
ALL
The most useful lab test in helping to diagnose ALL is… (be specific)
CBC w/ diff
What chromosome abnormalities are associated with ALL?
Hyperdiploidy + translocation of 12 and 21
Seen in the abnormal white cells
Phase 1 of ALL treatment is the () phase, which takes 15-18 months and achieves 95% remission on bone marrow aspirate using ()
- Remission induction phase
- Chemo for 15-18 months
Phase 2 in treating ALL, the intensification consolidation phase, adds on () to kill lymphoblasts in the (brain structure)
Adds on possible radiation to kill any lymphoblasts in meninges
Phase 3, the continuation therapy/maintenance phase of ALL treatment, uses daily () and weekly ()
- Daily oral chemo
- Weekly MTX
The main risk factor for ALL is…
Down syndrome
Always do a CBC on them!
The 2nd MC type of cancer in children is…
AML
Cancer of the bone marrow and lymph nodes
AML presents with very similar S/S as ALL. However, you can differentiate them via bone marrow aspirate showing () in AML and peripheral smear showing ()
- 30% or more blasts (ALL is 25)
- Auer rods (Pathognomonic for AML)
Mye rod? no its AUER rod!
I also remember it as america sounds like aml and auer! MC seen in 65+ as well.
AML treatment phases are similar to ALL, but the first phase is… (faster/slower)
Much faster! Its only 1 month of induction chemo for AML.
ALL is 15-18 months.
Besides treatment phases, AML can be treated via () or ()
Both transplants
- Bone marrow transplants
- Cord Blood transplants
The 3 RFs for AML are…
- () therapy
- Previous ()
- Syndromes: () or ()
- Ionizing radiation therapy
- Previous chemo
- Neurofibromatosis or Down syndrome
Remission is achieved in AML when () is in normal range
WBC
Between AML and ALL, the better prognosis/long-term survival is…
ALL
Hodgkin’s Lymphoma accounts for ()% of lymphomas in children, and primarily peaks right before () and after the age of ()
- 50% of lymphomas in children
- Peaks right before adolescence and again after age 50
Hodgkins in HS, Leukemia in little kids
The etiology of HL is that () cells undergo malignant transformation
Germinal center B cells
There are 3 forms of HL based on age: chilldhood, YA, and older adult.
Childhood form is characterized by INCREASED risk from () family size, and () socioeconomic status. Early exposure to () decreases risk!
Young adult form is INcreased risk from () socioeconomic status and being born (first vs last)
- Childhood risk = big family, poor status.
- Early exposure to Common infections decreases risk!
- Young adult risk = RICH and firstborn
Childhood vs young adult are opposite socioeconomic status for risk!
12yo M comes into your office with cervical LAN and supraclavicular LAN. It is painless. He has been having a cough, so you get a CXR and see a mediastinal mass. He has been losing a little weight recently and says he always wakes up drenched in the morning. Your primary suspicion is that this boy might have…
Hodgkin’s lymphoma
Biopsy of a lymph node shows Reed-Sternberg cells and EBV titer is positive. You know this corresponds to…
Hodgkin’s lymphoma
T/F: Hodgkin’s lymphoma is diagnosed via bone marrow aspirate and LP
False. It is to check if it has spread to bone marrow and spinal fluid. Dx is made via lymph node biopsy.
Reed-Sternberg cells are () lymphocytes
Multinucleated
You should early biopsy an enlarged lymph node in a child if:
- () of infectious cause
- Lymph node is > () cm
- () node is enlarged
- Abnormal ()
- Node increases in size after () weeks of abx tx or fails to decrease in 4-6 weeks.
- Lack of infectious cause
- > 2cm
- Supraclavicular (not a common node to get enlarged)
- Abnormal CXR
- 2 weeks of abx tx
Ann arbor staging is used for HL. The staging criteria and prognosis of HL correlates with ()
B symptoms
Fever, wt loss, night sweats
NHL arises from () cells and accumulates in () tissue
- Lymphoid cells
- Accumulates in lymph tissue
Name the Top 3 MC cancers in children.
- ALL
- AML
- NHL
L M N
NHL typically forms a tumor in… () , followed by the ()
- Abdominal tumor
- Chest tumor/mediastinal mass
Burkitt lymphoma/small noncleaved cell lymphoma is MC in () aged 5-10, originates from a () phenotype, MC has a tumor in the (), and is an abnormality of chromosome ()
- Boys 5-10 years old
- B-cell phenotype
- Intra-abdominal tumor
- Chromosome 8
8 Burkitt Boys Bellys
Chomosome 8
Burkitt Lymphoma
Boys 5-10
Belly (MC tumor location)
Lymphoblastic lymphoma is typically seen in () boys, originates in () cells, and the MC tumor is located in.. ()
- Teen boys
- T-cells
- Thorax (chest)
All the Ts! Because Lymphoblastic Lymphoma has a TWIN (two Ls)
Two Ls make a T if you use your imagination
NHL (lymphoblastic lymphoma) is INDISTINGUISHABLE from () unless you get this test ()
Indistinguishable from ALL. Must get a bone marrow aspirate showing > 25% blasts to know if it is ALL.
Lymphoblastic lymphoma is characterized by translocation of chromosome () and () + deletion of chromosome ()
- 14q11 and 7 switch
- 1 is deleted.
Large cell lymphoma, the rarest of the NHLs, is chacterized by Both B and T cells or histiocytes. The MC tumor location for it is….
Abdominal tumor
The Diagnosis of NHL is made via…
Biopsy + histology + tissue removal.
() is the tumor marker used to monitor NHL activity
LDH
The biggest RFs for getting NHL is those who have had organ (), (virus), (virus), and radiation exposure.
- Organ transplant
- EBV
- HIV
The classic triad associated with a brain/spinal tumor in a child is…
- morning ()
- ()
- ()
- Morning headache
- Vomiting
- Papilledema
The MC solid tumors of childhood are found in the…
Brain and spine
Diagnosis of a brain/spinal tumor is via
MRI w/ and w/o contrast
Generally, half of all brain/spinal tumors occur above () and the other half in the ()
- 50% above tentorium
- 50% in posterior fossa
Overall, the MC brain tumor of childhood is a (), which is located in the (). It is diagnosed via () and confirmed via ()
- Astrocytoma
- Cerebellum
- MRI to dx
- Biopsy to confirm.
Astrocytomas are graded WHO grades 1-4. The MC grade is (), which is treated via ().
WHO Grade I, requires surgical removal only.
Prior to removal of an astrocytoma, you want to administer () and () followed by ()
- Steroids and anticonvulsants prior to surgery
- High systemic chemo to follow
Brainstem Gliomas are difficult to treat because…
Middle of brainstem. Generally they are NOT removed.
This brain tumor is hard to treat and it is exclusively in school-aged children. It is probably a….
Brainstem glioma
Ependymomas can end up blocking ()
CSF flow
Optic Nerve Gliomas are common in what underlying condition?
Neurofibromatosis
The MC MALIGNANT brain tumor of childhood is the …, and commonly metastasizes to the ()
- Medulloblastoma
- Mets to the spinal cord
Medulloblastoma is Malignant like Medusa, who turns people to Stone (spinal cord) yay percy jackson
() is an EXTREMELY malignant neoplasm made of () tissue, but it is actually MC found in the ()
Neuroblastomas are made of nerve tissue, but usually found in the adrenal gland!
A 1y M patient is having bone pain and constitutional symptoms. Physical exam reveals a fixed, firm, immobile abdominal mass that extends past the midline. You suspect that is a () and decide to order a urinary () test
- Neuroblastoma
- Order urinary catecholamines (VMA/HVA
Vanilla acids
For a neuroblastoma to probably only need surgical removal for treatment, the patient is usually going to be (age) and the tumor should be (size)
Less than 1 year old with a small tumor
An abdominal tumor in childhood that does NOT cross the midline and is mobile/displaceable is most likely a ()
NEPHROblastoma
Neuro crosses.
Can be unilateral or bilateral!
A Wilm’s tumor is usually (symptoms) and is the (1st vs 2nd) MC abdominal tumor.
Asymptomatic and is 2nd MC abdominal tumor
The diagnosis of a Wilms tumor/nephroblastoma is first via () and then (). However, parents usually notice it first.
U/S first, then CT abdomen
Wilms tumor/nephroblastoma MC metastasis site is…
Lungs
Osteosarcoma MC affects the (bone section), while Ewing’s sarcoma affects the (bone section)
- Osteosarcoma = Metaphysis
- Ewing’s sarcoma = diaphysis
Osteosarcoma = Opposite ends. Ewing’s = Patrick Ewing was tall af
MC bone cancer in children/adolescents
Osteosarcoma
The 2 suppressor genes affected in cancer related to osteoblasts are…
p53 and Rb mutations
RB since osteosarcoma and retinoblastoma are common together?
The MC bone specifically affected in osteosarcoma is…
Distal femur
Long tubular bones
T/F: Osteosarcoma patients are typically taller than their peers
True
Besides checking the bones themselves in osteosarcoma, we should order a () because we fear mets to the ()
CT chest to check for pulmonary mets
Osteosarcoma has a strong genetic link with what other childhood cancer?
Retinoblastoma
Tx of osteosarcoma is via PO/IV () and ()
- Chemo
- Limb salvage
The XR findings suggestive of osteosarcoma are periosteal reactions, which may be written as () appearance or ()
- Sunburst appearance
- Codman’s triangle
Ewing’s sarcoma is often misdiagnosed as () and often presents with () at tumor site and ()
- Growing pains
- Soreness
- Swelling + warm to touch
Tx of Ewing’s sarcoma is via IV () prior to surgery for () months
IV Chemo for 6-9 months
Bone pain that wakes you up at night as a childhood + limp is probably either () or ()
- Osteosarcoma
- Ewing’s sarcoma
Retinoblastoma is a mutation in () gene in the long arm of chromosome ()
RB1 gene on chromosome 13
Retinoblastoma gene?
The MC finding associated with retinoblastoma is…
Leukocoria
Cat’s eye
May also see asymmetry of eyes, strabismus, pain
Mainstay of tx for retinoblastoma is…
External beam radiation
And removal of eye
Dx of retinoblastoma is via…
CT orbits
