BRS Oncology Flashcards
B and T cell dysfunction, atopic dermatitis, thrombocytopenia, assoc with leukemia and lymphoma
wiskott-aldrich syndrome
X-linked lymphoproliferative disease is associated with ______ infection and may result in ______
EBV
lymphoma
Burkitt’s lymphoma associated with _____ infection
Kaposi’s sarcoma associated with _____ infection
Hodgkin’s lymphoma
EBV
HIV
EBV
_____ is the most common childhood cancer
ALL
peak incidence and most likely demographic of ALL
2-6 years, male caucasian
classification of ALL cell morphology
L1, L2, L3 with L1 being the most common in childhood (small with little cytoplasm)
immunophenotypes of ALL
pre-B-cell: 70% –> 70% CALLA +, 30% CALLA -
T cell: 25%
B cell: < 5%
clinical features of ALL
fever, bone/joint pain
pallor, bruising, HSM, LAD
what does CBC show in ALL
anemia
thrombocytopenia
WBC is variable- leukemic blasts are often seen
how to confirm dx of ALL
bone marrow biopsy demonstrates marrow replacement by lymphoblasts
-then do cytogenetics
good prognostic factors in ALL
1-9 years of age
female
white
<50,000 WBCs
hyperploidy (more than 53 chromosomes in leukemic cells)
CALLA +
no organ involvement or chromosomal translocation
3 stages of treatment for ALL
- induction with steroids, vincristine, L-asparaginase and also intrathecal methotrexate**
- consolidation with intrathecal methotrexate +/- cranial irradiation
- daily and periodic chemo for up to 3 years if no sxs
when to do BMT for ALL
very high risk children
those who have relapsed
some complications of ALL and the tx for ALL
- infection 2/2 neutropenia: staph, e coli, pseudomonas
- opportunistic infections: fungi, PCP
- tumor lysis syndrome: hyperuricemia (renal probs), hyperkalemia (arrhythmias), hyperphosphatemia (hypocalcemia and tetany)
prognosis for ALL
generally good
85% long term survival
AML is often associated with ______
Down syndrome
classification of AML
M1-M7
M1, 2, 4, and 5 are most common
M3 (acute promyelocytic leukemia)- Auer rods
M7 (acute megakaryocytic leukemia)- associated with Down syndrome
which is CNS involvement more common?
AML vs. ALL
AML
clinical signs and sxs of AML
fever, HSM, bruising and bleeding, gingival hypertrophy, bone pain
LAD and testicular involvement are uncommon
lab findings of AML
pancytopenia or leukocytosis and DIC
how to dx AML
- blood smear shows leukemic myeloblasts esp with *Auer rods
- bone marrow biopsy for confirmation
how to tx AML
AML (unlike ALL) requires intensive myeloablative therapy to induce remission –> then BMT is highly recommended
prognosis of AML
chemo, BMT, assoc with Down syndrome
chemotherapy- 50% respond
BMT- 70% respond
if assoc with Down syndrome, very responsive to tx
2 types of CML (least common leukemia in kids)
adult-type: older children, teens, Philadelphia chromosome, t(9,22) BCR/ABL1 juvenile CML (JMML): infants and kids < 2 years, no Philadelphia chromosome
presentation of adult-type CML
nonspecific sxs:fatigue, weight loss, pain
massive splenomegaly
extremely high WBCs > 100,000
presentation of JMML (juvenile CML)
fever chronic eczema-like facial rash suppurative LAD petechiae and purpura moderate luekocytosis WBC <100,000; anemia, thrombocytopenia
how to tx CML
BMT and gleevec (imatinib)
prognosis of CML
adult type- biphasic course
JMML- often fatal; relapse in at least 50%
cancer of the antigen-processing cells found in the lymph nodes or spleen
Hodgkins lymphoma
Hodgkin’s lymphoma most common presentation
slow and indolent
painless LAD in the supraclavicular/cervical regions
relatively common to see systemic symptoms
reed stern berg cell is seen in _____
Hodgkin’s lymphoma
how to dx Hodgkin’s lymphoma
lymph node bx where you see reed stern berg cell
staging of Hodgkin’s lymphoma
stage I: single lymph node
stage II: on same side of diaphragm
stage III: both sides of diaphragm
stage IV: disseminated to one or more extra-lymphatic organs
how to tx hodgkin’s lymphoma
what are some complications of tx
chemo and XRT
- growth retardation
- secondary malignancies
- hypothyroidism
- male sterility (very common)
prognosis of Hodgkin’s lymphoma
stage I and II: >80% long term survival
60-70% for more advanced disease
non-hodgkin’s lymphoma is ______ (indolent/aggressive)
more male or female?
associated with _______ states
aggressive
male
immunodeficiency states (HIV, wiskott-aldrich, ataxia telengiectasia, prior EBV)
3 categories of non-Hodgkin’s lymphoma
- lymphoblastic lymphoma- T cell, histologically similar to ALL, anterior mediastinal mass, SVC syndrome or airway obstruction
- small non cleaved cell lymphoma such as burkitt’s- B cell, intussusception, jaw mass in burkitt’s
- large cell lymphoma- B cell, enlargement of lymphoid tissue in tonsils, adenoids, peyer’s patches
most common lymphoma in childhood
burkitt’s
most common presenting feature in non-Hodgkin’s lymphoma
painless LAD
suspect _______ in any child > 3 years presenting with intussusception
lymphoma- esp small non cleaved cell lymphoma
how to dx non-Hodgkin’s lymphoma
LN biopsy and imaging of various sorts for staging
how to tx non-Hodgkin’s lymphoma
treatment must be rapid
- surgery
- chemo
- ppx for CNS disease
- tx tumor lysis syndrome
after leukemia, _____ are the second most common childhood cancer
they are also the most common solid tumors in kids
brain tumors
4 most common brain cancers by histology
- glial cell tumors: astrocytomas
- primitive neuroectodermal tumors (PNET): medulloblastomas in the cerebellum
- ependymomas
- craniopharyngiomas
in kids, _______ (infratentorial/supratentorial) tumors are more common
infratentorial
MC infratentorial tumor
MC supratentorial tumor
medulloblastoma
astrocytoma
brain tumor: diminished vision, visual field deficits, strabismus
optic glioma
brain tumor: growth retardation, delayed puberty, visual changes, DI, other hormonal problems
craniopharyngioma
how to dx brain tumors
MRI
CSF analysis at the time of surgery
how to tx brain tumors
- surgery
- chemo
- try not to do XRT if under age 5 years
poorest prognosis with brain cancer
brainstem gliomas
not resectable
chemo doesn’t work
malignant tumor of neural crest cells arising anywhere along the sympathetic ganglia chain and within the adrenal medulla
neuroblastoma
peak incidence of neuroblastoma
age 2 years
most common presentation of neuroblastoma
firm abdominal mass that crosses the midline
how to dx neuroblastoma
-definitive dx by bone marrow bx + elevated urine catecholamines (VMA, HVA)
OR
-tissue biopsy
also CT or MRI for assessing tumor spread and then bone scan/skeletal survey to assess for mets to the bone
staging of neuroblastoma
I: tumor confined to structure of origin
II: extends beyond structure of origin but doesn’t cross midline
III: tumor goes past midline
IV: distant mets
IVS: localized tumor at stage I or II but distant mets to any organ but bone
how to tx neuroblastoma
surgery alone for stage I and II
chemo for metastatic disease and locally advanced dz
XRT for advanced disease
prognosis of neuroblastoma
good prognosis -children < 1 year -stage I and II disease -spontaneous regression can happen in young infants with stage IVS disease bad prognosis -stage III and IV disease -amplification of oncogene N-myc -high levels of ferritin, LDH, and neuron-speicfic enolase
______ is a childhood tumor of the kidneys
Wilm’s tumor (nephroblastoma)
peak incidence of wilm’s tumor
age 3 ears
hemihypertrophy, macroglossia, visceromegaly
beckwith-wiedemann syndrome
wilms tumor, aniridia, GU abnormalities, MR
WAGR syndrome
most common presentation of wilm’s tumor
abdominal mass (smooth, firm, rarely crosses midline)
abdominal pain
hematuria
HTN
how to dx wilm’s tumor
CT or MRI
bx and histology
how many stages of wilm’s tumor
I-V
V is bilateral renal involvement
how to tx wilm’s tumor
prompt surgery
chemo
XRT for advanced dz
prognosis of wilm’s tumor
excellent in most cases
most common soft tissue sarcoma in childhood
rhabdomyosarcoma- malignant tumor of the same embryonic mesenchyme that gives rise to skeletal muscle
common presentation rhabdomyosarcoma
painless soft tissue mass
most common areas involved in rhabdomyosarcoma
- head and neck
- orbital tumors- proptosis, chemosis, eyelid swelling, CN palsies
- nasopharyngeal tumors- epistaxis, airway obstruction, chronic sinusitis
- laryngeal tumors- hoarseness - GU tract- hematuria, urinary tract obstruction, abd mass
- extremities- painless growing mass
how to dx rhabdomyosarcoma
CT or MRI
bx and histology
how to tx rhabdomyosarcoma
surgical resection
+/- chemo and XRT
prognosis of rhabdomyosarcoma
which sites have the best prognosis
head and neck and GU tumors have the best prognosis
most common malignant bone tumor
peak incidence and gender
osteogenic sarcoma
male teens
most likely location for osteogenic sarcoma
near the knee
*metaphyses of tubular long bones
systemic sxs are _______ common/uncommon in osteogenic sarcoma
how about ewing’s sarcoma
uncommon with osteogenic sarcoma
common in ewing’s sarcoma (fever, malaise, weight loss)
X-ray finding in osteogenic sarcoma and ewing’s sarcoma
osteogenic- sunburst
ewing’s- onion skin
most common locations for ewing’s sarcoma
flat bones and diaphysis of tubular bones; most commonly in axial skeleton (esp pelvis)
how to dx primary bone cancer (osteogenic and ewing’s sarcoma)
MRI –> tissue biopsy –> bone scan, chest CT and other stuff to look for mets
how to tx osteogenic sarcoma
surgery for primary lesion and pulmonary mets
chemo
prognosis of osteogenic sarcoma
survival > 60%
bone cancer with small, ground, blue cell tumor
ewing’s sarcoma
second most common malignant bone tumor occurring in male adolescents
ewing’s sarcoma
chromosomal translocation in ewing’s sarcoma
t (11,21)
how to tx ewing’s sarcoma
prognosis
multiagent chemo followed by surgical excision
XRT if complete excision is not possible
-prognosis good for local dz, poor if mets are present
most common liver tumor in childhood
what age group
what is it assoc with
hepatoblastoma
< 3 years
beckwith-wiedemann syndrome
hepatocellular carcinoma in childhood is most likely 2/2 _____
chronic active hep B infection
also biliary atresia, glucogen storage disease type I, alpha-1-antitrypsin deficiency, hereditary tyrosinemia
presentation of liver tumors in childhood
RUQ mass, loss of appetite and weight
jaundice is usually absent
how to dx liver tumors in childhood
CT or MRI
look for elevated alpha-fetoprotein in the serum
how to tx liver tumors in childhood
what’s the prognosis
chemo and surgical resection
overall, prognosis is quite poor and often end up with mets
\_\_\_\_\_\_ is the most common teratoma during first year of life female vs. male what's the presentation benign or malignant how to tx
sacrococcygeal female predominant soft tissue mass growing from coccyx benign tx with surgical excision
anterior mediastinal teratomas are generally _______ (benign/malignant) and can present with ______
benign
airway obstruction
ovarian teratomas are the most common ovarian tumor and are generally _____ (benign/malignant)
you may see ______ on abdominal xray
benign
calcium in the tumor
most common testicular tumors
yolk sac tumor > teratoma
peak ages of testicualr tumor
< 5 years and during teen years
in kids, chances of testicular tumor being benign
what about adults
in kids, 1/3 are benign
in adults, all are malignant
elevated alpha fetoprotein is seen in which germ cell tumor
yolk sac tumor
for ovarian tumors, 1/3 are malignant
the younger the child is, the more likely that the tumor is ____ benign/malignant
malignant
eosinophilic granuloma
Hand-Schuller-Christian disease
Letterer-Siwe disease
all are part of Langerhans Cell Histiocytosis (LCH)
-severe immune dysregulation disease that may be cancer-like
common clinical involvement with langerhans cell histiocytosis (LCH)
- skeletal involvement- skull, bony lesions, chronic draining ears indicates mastoid LCH
- skin involvement- seborrheic dermatitis of the diaper area and scalp
- pituitary or hypothalamic involvement
how to dx LCH
histologic features on skin or bone bx
how to tx LCH
prognosis
if single lesion or organ –> local curettage, low dose XRT, steroids, or single agent chemo
if multiple lesions –> multi agent chemo
prognosis is variable based on extent of disease
Down syndrome puts you at risk for these cancers
ALL and AML
beckwith-wiedemann syndrome predisposes to these tumors
wilms, rhabdomyosarcoma, hepatoblastoma
neurofibromastosis type I predisposes to these tumors
brain tumors and lymphoma
