Unusual Tumors in Children (under construction) Flashcards
Germ Cell Tumors in Children
The risk of GCT decreased among female
cases with a family history of cancer with onset before 40 years.
TRUE or FALSE?
False
The Children’s Oncology
Group evaluated the link between a family history of cancer and pediatric GCT
in a case–control study of 274 cases. The risk of GCT decreased among female
cases with a family history with onset before 40 years but increased for boys,
especially for those with a family history of melanoma.
Germ Cell Tumors in Children
What is he most frequent
cytogenetic abnormality for both gonadal and
extragonadal GCT, except for children <3 years of age at diagnosis?
isochromosome 12p
Germ Cell Tumors in Children
Embryonal carcinoma
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
\+ \+ – \+ _
(b-hcg, cea, afp)
em”B”ryonal “CA”
Germ Cell Tumors in Children
Mature teratoma
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
– – – – –
none
Germ Cell Tumors in Children
Immature teratoma
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
\+ \+ \+ \+ –
all except PLAP
Germ Cell Tumors in Children
Choriocarcinoma
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
\+++ – \+ \+ –
B-HCG and LDH
Germ Cell Tumors in Children
Yolk sac tumor
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
– \+++ \+ \+ –
AFP, LDH, CEA
yo”L”k s”AC” tumor
Germ Cell Tumors in Children
Germinoma
Indicate + or +++ or (-) for the following serum markers:
B-HCG AFP LDH CEA PLAP
\+ – \+ \+ \+++
no afp
Germ Cell Tumors in Children
Most common extracranial presenting signs/symptoms
Abdominal pain
abdominal distension
and sacrococcygeal or buttock swelling
Germ Cell Tumors in Children
Half-time of AFP
5 to 7 days
Germ Cell Tumors in Children
Half-time of B-HCG
24 to 36 hours (1-2 days)
Germ Cell Tumors in Children
An infant less than 1 year old presenting with high levels of AFP (>1,000 mg/mL) should raise a clinical suspicion of a _______ tumor.
NONE
that level is normal in infants < 1 month
Germ Cell Tumors in Children
Lymph node
dissection is recommended in early-stage gonadal GCT because of the high incidence of involvement.
TRUE or FALSE?
False
Germ Cell Tumors in Children
There is no indication for
radiotherapy after complete response in extragonadal GCT.
TRUE or FALSE?
True
NPC in Childhood
NPC is 6% to 18% of all cases in non-endemic regions, compared to <1% to 2% in endemic regions.
TRUE or FALSE?
False
Interestingly, although the absolute numbers are lower in
nonendemic countries (United States, Turkey, Israel), childhood NPC is 6% to
18% of all cases compared to <1% to 2% in endemic countries/regions (southern
China, Southeast Asia, Mediterranean basin).
NPC in Childhood
As in older patients, Epstein-Barr virus is associated with this type of NPC in addition to what certain human leukocyte antigen (HLA) haplotypes/alleles?
A2 Bsin2, Aw19, Bw46, and B17
NPC in Childhood
What is the typical initial presentation?
painless upper neck mass
JNA
Identify the stage:
Limited to the nose and/or nasopharynx
Ia
JNA
Identify the stage:
Minimal extension through the sphenopalatine foramen, into and including a minimal
part of the medialmost part of pterygomaxillary fossa
IIa
JNA
Identify the stage:
Extension through the pterygomaxillary fossa into the cheek and temporal fossa or
posterior to the pterygoid plates
IIc
JNA
Identify the stage:
extension into one or more paranasal sinuses
Ib
JNA
Identify the stage:
Full occupation of the pterygomaxillary fossa, displacing the posterior wall of the
maxillary antrum forward; lateral and/or anterior displacement of branches of the
maxillary artery; superior extension may occur, eroding orbital bones
IIb
JNA
Identify the stage:
Erosion of the skull base with minimal intracranial extension
IIIa
JNA
Identify the stage:
Erosion of the skull base with extensive intracranial extension with or without
cavernous sinus invasion
IIIb
