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Ph RadOnc Residency PBRROE2-3 Review 2021 > Unusual Tumors in Children (under construction) > Flashcards

Unusual Tumors in Children (under construction) Flashcards

1
Q

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?

A

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.

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2
Q

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?

A

isochromosome 12p

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3
Q

Germ Cell Tumors in Children

Embryonal carcinoma

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
\+
\+
–
\+
_

(b-hcg, cea, afp)
em”B”ryonal “CA”

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4
Q

Germ Cell Tumors in Children

Mature teratoma

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
–
–
–
–
–

none

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5
Q

Germ Cell Tumors in Children

Immature teratoma

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
\+
\+
\+
\+
–

all except PLAP

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6
Q

Germ Cell Tumors in Children

Choriocarcinoma

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
\+++
–
\+
\+
–

B-HCG and LDH

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7
Q

Germ Cell Tumors in Children

Yolk sac tumor

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
–
\+++
\+
\+
–

AFP, LDH, CEA

yo”L”k s”AC” tumor

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8
Q

Germ Cell Tumors in Children

Germinoma

Indicate + or +++ or (-) for the following serum markers:

B-HCG
AFP
LDH
CEA
PLAP
A 
\+
–
\+
\+
\+++

no afp

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9
Q

Germ Cell Tumors in Children

Most common extracranial presenting signs/symptoms

A

Abdominal pain
abdominal distension
and sacrococcygeal or buttock swelling

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10
Q

Germ Cell Tumors in Children

Half-time of AFP

A

5 to 7 days

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11
Q

Germ Cell Tumors in Children

Half-time of B-HCG

A

24 to 36 hours (1-2 days)

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12
Q

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.

A

NONE

that level is normal in infants < 1 month

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13
Q

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?

A

False

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14
Q

Germ Cell Tumors in Children

There is no indication for
radiotherapy after complete response in extragonadal GCT.

TRUE or FALSE?

A

True

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15
Q

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?

A

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).

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16
Q

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?

A

A2 Bsin2, Aw19, Bw46, and B17

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17
Q

NPC in Childhood

What is the typical initial presentation?

A

painless upper neck mass

18
Q

JNA
Identify the stage:

Limited to the nose and/or nasopharynx

A

Ia

19
Q

JNA
Identify the stage:

Minimal extension through the sphenopalatine foramen, into and including a minimal
part of the medialmost part of pterygomaxillary fossa

A

IIa

20
Q

JNA
Identify the stage:

Extension through the pterygomaxillary fossa into the cheek and temporal fossa or
posterior to the pterygoid plates

A

IIc

21
Q

JNA
Identify the stage:

extension into one or more paranasal sinuses

A

Ib

22
Q

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

A

IIb

23
Q

JNA
Identify the stage:

Erosion of the skull base with minimal intracranial extension

A

IIIa

24
Q

JNA
Identify the stage:

Erosion of the skull base with extensive intracranial extension with or without
cavernous sinus invasion

A

IIIb

25
Q

Unusual Tumors in Children
Hemangiomas

(bonus. this is already in benign diseases chapter)

A

(bonus. this is already in benign diseases chapter)

26
Q

Unusual Tumors in Children
Esthesioneuroblastoma

(bonus. this is already in the adult’s chapter since management is the same)

A

(bonus. this is already in the adult’s chapter since management is the same)

27
Q

Unusual Tumors in Children
Oropharynx and Salivary Glands

(bonus. this is already in the adult’s chapter since management is the same)

A

(bonus. this is already in the adult’s chapter since management is the same)

28
Q

Unusual Tumors in Children

What is the more common histology in pediatric bronchogenic carcinoma?

A

Undifferentiated

Unusual Tumors in Children

29
Q

Unusual Tumors in Children

What is the most common primary malignancy of the lung in childhood.

A

PPB

pleuropulmonary blastoma

30
Q

Unusual Tumors in Children

What is the main histologic difference between pulmonary blastoma and PPB?

(PPB is purely a pediatric tumor)

A

absence of epithelial carcinomatous components in PPB;

PPB consists of mesenchymal stroma only.

31
Q

Unusual Tumors in Children

What germ-line mutation is the known cause of the majority of PPB cases?

A

mutation in DICER1

32
Q

Unusual Tumors in Children

Give two most common histologies of breast cancer in the pediatric age group.

A

adenocarcinoma

cystosarcoma phyllodes

33
Q

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

A

Juvenile secretory carcinoma

34
Q

Unusual Tumors in Children
LCH

Positive staining for ____ and _____ are rquired for the diagnosis.

A

CD1a

CD207 (langerin)

35
Q

Unusual Tumors in Children
LCH

Classic electron microscopic finding

A

Birbeck granules (langerin expressing cytoplasmic organelles)

36
Q

Unusual Tumors in Children
LCH

What is the Lahey criteria?

A

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

37
Q

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)

A

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.

38
Q

Unusual Tumors in Children
ACC

ACC is associated with what syndrome and hemihypertrophy?

A

Beckwith-Wiedemann syndrome

39
Q

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?

A

virilization

40
Q

Unusual Tumors in Children
ACC

Steroid production is limited by ACTH suppression

TRUE or FALSE?

A

False

41
Q

Unusual Tumors in Children
ACC

What is the characteristic ultrasound findings?

A

echo star pattern

Ph RadOnc Residency PBRROE2-3 Review 2021 (57 decks)

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