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Peds Flashcards

1
Q

What is the most common supratentorial pediatric tumor?

A

Astrocytoma

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2
Q

What is typical for Diffuse hemispheric glioma, H3 G34 mutant?

A
  • very rare and aggressive.
  • Histologically primitive embryonal appearance.
  • Adolescents and young adults.
  • Prognosis comparable to Glioblastoma, IDH wt.
  • Imaging looks like glioblastoma. But calcifications may also be seen. INTERESTINGLY do not exhibit peritumoral edema.
  • Local recurrence and leptomeningeal dissemination is possible.
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3
Q

4 clinical signs of pediatric brain tumor

A

Vomiting
Arrest of development
Failure to thrive
Seizures

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4
Q

PNETS are called medulloblastoma in a specific location. What location?

A

posterior skullbase

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5
Q

What type of tumor is medulloblastoma? Give another name for it.

A

Its an embryonal type of tumor. Another name is PNET - primitive neuroectodermal tumor

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6
Q

What does PNET stand for?

A

Primitive neuroectodermal tumor

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7
Q

Name 5 types of PNETs

A

Medulloblastoma
Pineoblastoma
Neuroblastoma
Esthesioneuroblastoma
Retinoblastoma

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8
Q

What is Collins law?

A

Risk of recurrence is
Age at diagnosis + 9 months.

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9
Q

Which are the two most common (in the right order) intralmedullary (spinal) tumors in children?

A
  1. astrocytomas
  2. ependymomas
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10
Q

Pediatric gliomas are recognised as distinct from their adult counterparts and got their own classification. Why?

A
  • different genomic alterations
  • different biological behaviour
  • different clinical course
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11
Q

What are the two pediatric categories found under “Gliomas, glioneuronal tumors and neuronal tumors”?

A
  • pediatric-type diffuse low -grade glioma
  • pediatric type high-grade glioma.
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12
Q

What is typical for pediatric glioblastoma?

A

It doesnt exist. The characteristic alterations seen in glioblastoma are not seen in pediatric cases and is therefore considered an adult tumor.

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13
Q

Is pilocytic astrocytoma a pediatric tumor?

A

No, its mostly seen in children, but its not pediatric-only and is found under “circumscribed astrocytic gliomas”.

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14
Q

Why can imaging be crucial for accurate diagnostics?

A

For instance, diffuse and circumscribed gliomas need to be distinguished by imaging as the distinction can be difficult on pathology, especially in small samples.

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15
Q

So! New alterations to know for children.
Which are the low grade gliomas?

A
  • Diffuse astrocytoma, MYB or MYBL1 altered
  • Angiocentric glioma
  • Polymorphous low-grade neuroepithelial tumor of the young
  • Diffuse low-grade glioma, MAPK pathway altered.
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16
Q

Which are the pediatric type diffuse high grade gliomas?

A
  • diffuse hemispheric glioma H3 G34 mutant
  • Diffuse pediatric type high grade glioma, H3-wt and IDH-wt
  • Diffuse midline glioma, H3 K27 altered
  • Infant type hemispheric glioma
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17
Q

What is typical for Diffuse midline glioma H3 K27 altered?

A
  • Grade 4 regardlless of histology
  • Midline location. Typically involves pons. - also called diffuse intrinsic pontine glioma.
  • less than 10% 2-year survival
  • Expansile infiltrative tumor centered in pons with varying degrees of cranial or caudal extension.
  • Growth into prepontine cistern and engulfment of basilar artery is common.
  • Obs! Enhancement is abscent or mild and heterogenous! Increases over time and is not a good sign.
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18
Q

How common is it that pediatric gliomas do not have H3 or IDH mutations?

A

1/3.

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20
Q

What is typical for pediatric type high grade glioma H3 wt, IDH wt?

A
  • all ages of childhood.
  • mostly supratentorial
  • 3 major molecular entities:
    1- MYCN ampl - most common and most aggressive. 26% 3 year survival.
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20
Q

What are the differences between Diffuse hemispheric glioma H3 G34 mutant and Diffuse midline glioma H3 K27 altered?

A
  • The H3 K27 midline glioma is situated MIDLINE.
  • It has far worse outcome
  • The H3 mutations differ H3 G34 and H3 K27.
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21
Q

Why are high grade astrocytomas with piloid features discussed as pediatric tumors when they are seen in all ages?

A

10% of the high grade astrocytomas with piloid features are seen in children.

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22
Q

Where are high grade astrocytomas with piloid features usually seen?

A

In cerebellum. 74%.

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23
Q

How is the prognostics for high grade astrocytomas with piloid features compared to conventional pilocytic astrocytomas and IDH-mutant grade 3 astrocytomas?

A

Although abscence of high-grade findings on histology, the high grade astrocytomas with piloid features have shorter survival than conventional pilocytic astrocytomas and IDH-mut grade 3 astrocytomas.
It is similar to IDH mut grade 4 astrocytomas and better than IDH wt glioblastoma.

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24
Q

Prognosis of high grade astrocytomas with piloid features seems to be dependent on age. How?

A

Better outcomes in pediatric cases.

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25
Q

Describe Diffuse astrocytoma, MYB or MYBL1 altered

A
  • WHO grade 1 median age 5 yo.
  • Supratentorial usually Temporal site.
  • Long standing EP MOST COMMON presentation.
  • Majority survive 5 years and cure/improvement of EP after surgery.
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26
Q

Describe Polymorphous low-grade neuroepithelial tumor of the young

A

PLNTY
SPECIAL: The amount and pattern of calcification is unusual for other pediatric brain tumors.
+ cystic components in 40-89%. +/- enhancement.

  • grade 1 diffuse gliomas
  • Typically refractory EP in children/young adults.
  • Typically temporal lobe
  • BRAF mutations
  • Fusions of FGFR

! Surgery improves seizures in most cases and recurrence is unlikely.
* Can be well circumscribed or have unclear boundaries. ‘

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27
Q

Describe diffuse low grade glioma, MAPK-pathway altered

A
  • median age 6-11 yo
  • commonly associated w seizures
  • hemispheric although 26% in Diencephalon.
  • If FGFR mutated, rapid progression is seen in 50%.
  • All are indolent and death occurs MORE than 10 years after diagnosis.
  • Rarely transform.
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28
Q

Pediatric ependymomas, just like adult ependymomas, are different in different locations. Describe the distribution of ependymomas in children in the three sites.

A

27% supratentorial.
73% posterior fossa
10% spinal.

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29
Q

So, in children, posterior fossa ependymoma are the most common. They exist in 2 subtypes. Which?

A

PFA and PFB - posterior fossa A and B.

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30
Q

What differs between posterior fossa ependymoma type A and B?

A

A are younger - children under 5 “exclusively” show A.
50% teenagers = Type B.

A= Higher incidence of recurrence and mortality.

B= Excellent outcome. many cured w GTR alone.
B has 50% recurrence after 5 years.

A= arise more lateral.
B tend to occur in the midline. (4th ventricle)

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31
Q

What is the most likely diagnosis?
- A 3 yo girl with a posterior fossa tumor that is situated laterally in the cerebellar hemisphere

A

Ependymoma type A

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32
Q

Pediatric Midline posterior fossa tumors:

A
  • Ependymoma type B -teenagers, 4th ventricle
    *circumscribed astrocytoma
  • diffuse midline gliomas - typically involves pons - 7 yo
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33
Q

What is DNET?

A
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34
Q

How is the prognostics for high grade astrocytomas with piloid features compared to conventional pilocytic astrocytomas and IDH-mutant grade 3 astrocytomas?

A

Although abscence of high-grade findings on histology, the high grade astrocytomas with piloid features have shorter survival than conventional pilocytic astrocytomas and IDH-mut grade 3 astrocytomas.
It is similar to IDH mut grade 4 astrocytomas and better than IDH wt glioblastoma.

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35
Q

What is the 1st and 2nd most common malignant brain tumor of childhood?

A

1st = high grade gliomas.
2nd = medulloblastomas.

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36
Q

How does medulloblastomas present?

A
  • midline masses
  • roof of 4th ventricle
  • associated masseffect
  • hydrochephalus
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37
Q

What is the normal treatment of medulloblastoma in children?

A
  • surgical resection
  • radiation therapy
  • chemotherapy
38
Q

What influences the prognosis for medulloblastomas?

A
  • grade of surgical resection
  • precence of CSF mets at the time of diagnosis
  • expression of HER2 oncogene and other molecular markers!! (4 groups)
39
Q

What are the 4 groups of medulloblastomas?

A
  • WNT
  • SHH
  • group3
  • group4
40
Q

Which is the most difficult differential diagnosis to medulloblastoma radiologically?

A

Atyical teratoid/rhabdoid tumors. (AT/RT)

41
Q

Typical presentation of AT/RT?

A
  • WHO gr 4
  • children under 2 y/o
  • 50% posterior fossa
  • very aggressive
    Surgery with debulking can be offered in some cases. Little if any response to chemotherapy. Death within a year.
42
Q

Which is the most common type of brainstem glioma in children?

A

Diffuse midline glioma H3 K27-altered.

43
Q

Where are diffuse midline gliomas usually situated in the infratentorial area?

A

Pontine.

44
Q

What are usual findings at presentation of brainstem gliomas?

A
  • ataxia
  • CRANIAL NERVE PALSIES
  • LONG TRACT SIGNS
  • hcph.
45
Q

How long history is typical for diffuse midline glioma, H3 K27altered?

A

days!

46
Q

What is the overall 5 year survival of pontine gliomas?

A

10%

47
Q

What is usually the treatment of diffuse midline glioma H3 K27 altered?

A

Radiation.
surgery usually not possible.

48
Q

What is the most common supratentorial pediatric tumor?

A

Astrocytoma

49
Q

It has been belived that most pediatric tumors are infratentorial (60%) but it seems to be dependent on something. what?

A

Age. It varies a lot between age groups.

50
Q

What are the three major infratentorial tumors in children?

A
  • medulloblastoma (PNET)
  • cerebellar astrocytoma
  • ependymoma
51
Q

What is the most common location for braintumors in children up to 3 yo?

A

Supratentorial.

52
Q

What is the most common location of braintumors in ages above 10 y/o?

A

supratentorial.

53
Q

What type of origin of tumors does younger children usually have?

A

embryonal origin.

54
Q

What type of origin of tumors does older children usually have?

A

Glial origin.

55
Q

Which is the most common supratentorial tumor in all ages children-grown ups?

A

Astrocytomas.

56
Q

How common are pineal tumors in children?

A

3-11% of all pediatric brain tumors.

57
Q

There are several types of Pineal tumors. Which are the 2 most interesting?

A
  • Germ cell tumors
  • pineal parenchymal cell t
58
Q

Except for hcph, what special traits must be looked for in case of pineal tumors?

A
  • Evaluation of the pituitary function.
  • Visual field examination
59
Q

There are three types of germ cell tumors, which?

A
  • Teratomas
    *Germinomas
    *NGGCTs
60
Q

Name at least 2 pineal parenchymal tumors

A
  • Pineocytoma
  • Pineoblastoma
  • Pineal parenchymal tumors of intermediate differentiation
  • Pineal papillary tumors
61
Q

Why should CSF and serum be measured for certain markers when a pineal tumor is found?

A

Alpha fetoprotein and beta human chorionic gonadotropin are pathognomonic for certain germ cell tumors.

62
Q

Why is it important if it is a germ cell tumor?

A

No need for surgery, chemotherapy and radiation can be started immediately.

63
Q

Why is it important if it is a germ cell tumor?

A

No need for surgery, chemotherapy and radiation can be started immediately.

64
Q

What is typical for pediatric type high grade glioma H3 wt, IDH wt?

A
  • all ages of childhood.
  • mostly supratentorial
  • 3 major molecular entities:
    1- MYCN ampl - most common and most aggressive. 26% 3 year survival.
65
Q

What is typical for pediatric type high grade glioma H3 wt, IDH wt?

A
  • all ages of childhood.
  • mostly supratentorial
  • 3 major molecular entities:
    1- MYCN ampl - most common and most aggressive. 26% 3 year survival.
66
Q

How common are pediatric pineal gland tumors?

A

10% of all ped brain tumors

67
Q

What can make it possible not to need even to do a biopsy of the pineal tumor?

A

If beta hCG or alfa fetoprotein is found in likvor and/serum in the child, its pathogmomonic for certain germ cell tumors and radiotherapy and chemotherapy is the choice of treatment.

68
Q

Which type of germ cell tumors are very radio-sensitive and which are more aggressive?

A

Germinomas are among the most radiosensitive tumors. NGGCTs (non.germinomatous germ cell tumors) such as choriocarcinoma and yolk sac tumors are more aggressive and chemotherapy and extensive craniospinal irradiation is needed.

69
Q

Teratomas are different from other germ cell tumors of the pineal gland in terms of treatment. How?

A

If they can be gross total resected, they may not need any other treatment. Otherwise they are treated as NGGCTs.

70
Q

Germ cell tumors are treated primarily with radiotherapy and chemo. The other type of pineal gland tumors are pineal parenchymal tumors. Is surgery needed for pineal parenchymal tumors?

A

Yes, all of them need surgical resection. Some, like pineoblastoma, are aggressive and need also CSI (craniospinal irradiation) and chemo.

71
Q

What is the craniopharyngeoma seen in children called?

A

Adamantinomatous craniopharyngeaoma.

72
Q

What WHO grade is adamantinomatous craniopharyngeoma have?

A

grade 1

73
Q

Where is adamantinomatous craniopharyngeomas normally seen and how does it present?

A

In the pituitary region (supracellar). Typically as a cystic mass with peripheral calcifications.

74
Q

What is the 3 year survival rate in children from craniopharyngeoma?

A

95%

75
Q

Differential diagnostics to craniopharyngeoma?

A
  • Rathkes cleft cysts (nothing solid, enhancing or calcifications and mostly intrasellar)
  • Pituitary macroadenoma (similar look but usually intrasellar)
  • Pituitary apoplexy (acute)
  • Intracranial teratoma (precense of fat)
76
Q

Parinaud syndrome may be seen on presentation of extraxial teratomas. What is that?

A
  • sunset sign - upward gaze palsy
  • convergence-retraction nystagmus
  • pupillary light-near dissociation (respond to near stimuli but not light)
77
Q

When does teratomas present dependent on type and where are they localized?

A
  • intra-axial - LARGE, typically cause problem in labour. More common supratentorially.
  • extra-axial - present in childhood/early adulthood. Typically smaller and in the pineal or suprasellar region.
78
Q

Although Teratomas are uncommon in the general population, they account for the largest population of fetal intracranial neoplasms. How many percent of fetal brain tumors are Teratomas?

A

26-50%!!

79
Q

Why is it such a serious sign w perinaud syndrome?

A

It suggests impingement to the tectal plate. Usually to a posterior commissure or pineal region solid mass rather than a cyst.

80
Q

What does DNET mean?

A

Dysembryoblastic neuroepithelial tumors.

81
Q

What grade (WHO) does DNET have?

A

grade 1

82
Q

Where in the brain are DNETs situated?

A

The vast majority in the cortical grey matter. 80% are associated with cortical dysplasia. Usually temporal.

83
Q

What type of clinical situation is associated to DNETs?

A

Intractable focal seizures.

84
Q

DNETs are usually diagnosed after onset of seizures. What age?

A

under 20y/o

85
Q

What syndrome is associated to DNETs?

A

Noonan syndrome.

86
Q

According to radiopedia 2023, what are the 4 most common tumors in the posterior fossa in children?

A
  • medulloblastoma
  • pilocytic astrocytoma
  • brainstem glioma
  • ependymoma
  • AT/RT
86
Q

Radiopedia 2023 has listed 3 supratentorial pediatric tumors in their pediatric core curriculum. Which?

A
  • Astrocytoma - pediatric-type diffuse low-grade gliomas, -pediatric-type diffuse high-grade gliomas and -circumscribed astrocytic gliomas ( in which pleomorphic xanthoastrocytoma belongs)
  • DNET
  • Pleomorphic xanthoastrocytoma - see Astrocytoma_circumscribed astrocytic glioma
87
Q

What is “SGNE”?

A

“specific glioneuronal element (SGNE)” is characteristic, and refers to columnar bundles of axons surrounded by oligodendrocyte-like cells which are oriented at right angles to the overlying cortical surface. Between these columns are “floating neurons” as well as stellate astrocytes.

88
Q

What is the Blumcke classification of cortical dysplasia?

A
89
Q

How are DNETs distinguished from low-grade astrocytomas and oligodendrogliomas (radiopedia info)?

A
  • IDH wt
  • p53 wt
  • no 1p19q co-deletion
90
Q

Differential diagnostics of DNETs?

A
  • Ganglioglioma - uncommon
  • Pleomorphic xanthoastrocytoma - contrast enhancement prominent.
  • dural tail often seen.
  • Oligodendroglioma RARE in children. (- IDH mut and 1p19q codeleted)

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