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
Unusual Tumors in Children
Hemangiomas
(bonus. this is already in benign diseases chapter)
(bonus. this is already in benign diseases chapter)
Unusual Tumors in Children
Esthesioneuroblastoma
(bonus. this is already in the adult’s chapter since management is the same)
(bonus. this is already in the adult’s chapter since management is the same)
Unusual Tumors in Children
Oropharynx and Salivary Glands
(bonus. this is already in the adult’s chapter since management is the same)
(bonus. this is already in the adult’s chapter since management is the same)
Unusual Tumors in Children
What is the more common histology in pediatric bronchogenic carcinoma?
Undifferentiated
Unusual Tumors in Children
Unusual Tumors in Children
What is the most common primary malignancy of the lung in childhood.
PPB
pleuropulmonary blastoma
Unusual Tumors in Children
What is the main histologic difference between pulmonary blastoma and PPB?
(PPB is purely a pediatric tumor)
absence of epithelial carcinomatous components in PPB;
PPB consists of mesenchymal stroma only.
Unusual Tumors in Children
What germ-line mutation is the known cause of the majority of PPB cases?
mutation in DICER1
Unusual Tumors in Children
Give two most common histologies of breast cancer in the pediatric age group.
adenocarcinoma
cystosarcoma phyllodes
Unusual Tumors in Children
What is a rare breast cancer that seemed to have a benign clinical course?
It is characterized by abundant mucin- and MPS-containing materials.
Hormone receptors are generally (-)
treated with excision alone
Juvenile secretory carcinoma
Unusual Tumors in Children
LCH
Positive staining for ____ and _____ are rquired for the diagnosis.
CD1a
CD207 (langerin)
Unusual Tumors in Children
LCH
Classic electron microscopic finding
Birbeck granules (langerin expressing cytoplasmic organelles)
Unusual Tumors in Children
LCH
What is the Lahey criteria?
Risk organs and their involvement were defined according to modified Lahey
criteria as follows:
Hematopoietic: Anemia and/or leukopenia and/or thrombocytopenia
Liver: Enlargement >3 cm below the costal margin, dysfunction, or both
Spleen: Enlargement >2 cm below the costal margin
Lung: Typical changes via high-resolution CT, histopathologic diagnosis, or
both
Unusual Tumors in Children
LCH
RT use has decreased.
What are the indications for RT to bony sites? (this is from adult benign neoplasms chapter)
Indications for RT to bony sites include relapse after surgery,
no signs of clinical healing after other interventions,
pain relief, potential compromise of critical structures from an expansile lesion (i.e., cord compression),
or if the bony site is not amenable to other local therapies.
Unusual Tumors in Children
ACC
ACC is associated with what syndrome and hemihypertrophy?
Beckwith-Wiedemann syndrome
Unusual Tumors in Children
ACC
Children usually present with abdominal pain and palpable mass.
In females, what is the most common presentation of functional tumors?
virilization
Unusual Tumors in Children
ACC
Steroid production is limited by ACTH suppression
TRUE or FALSE?
False
Unusual Tumors in Children
ACC
What is the characteristic ultrasound findings?
echo star pattern
