SFP: pediatric tumors Flashcards

(42 cards)

1
Q

What is heterotopia?

A

Normal tissue where it doesn’t belong.

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2
Q

What is an example of heterotopia?

A

Meckel’s diverticula.

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3
Q

What is hamartoma?

A

Right tissue, right place, but too much of it.

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4
Q

What is the most common tumor of infancy?

A

Hemangioma.

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5
Q

Describe the general presentation of hemangiomas.

A

Often on the skin, face, and scalp and usually spontaneously regress. They may be disfiguring.

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6
Q

What do hemangiomas look like on histology?

A

Lots of channels of benign blood vessels.

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7
Q

What is a lymphangioma?

A

Benign overgrowth of lymphatic channels, often occurring on the skin but may be deeper and imitate an invasive tumor.

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8
Q

What does lymphangioma look like on histology?

A

Just a bunch of lymphatic channels.

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9
Q

What is sacrococcygeal teratoma?

A

A germ cell tumor that can contain endoderm, mesoderm, and ectoderm.

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10
Q

Are sacrococcygeal teratomas benign or malignant?

A

Most are benign, but depends on maturity of the teratoma.

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11
Q

What is the difference between mature and immature teratoma on histology?

A

Mature have defined differences and characteristics, where immature is just a whole bunch of immature blue cells with no real differentiation.

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12
Q

What are the most common malignant tumor categories in kids?

A

Hematopoietic, CNS, soft tissue.

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13
Q

Describe neuroblastoma.

A

Malignant neural tissue tumors often found in the adrenal gland or spine that may spontaneously regress or mature.

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14
Q

How do we pathologically differentiate neuroblastoma?

A

MKI, differentiation, stroma, patient age.

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15
Q

How do we clinically stage neuroblastoma?

A

Location, completeness of excision, patient age.

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16
Q

What is a bad prognostic indicator if neuroblastoma?

A

MYCN amplification.

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17
Q

What is MYCN gene?

A

Proto-oncogene; amplification indicates poor prognosis for neuroblastoma.

18
Q

What does MYCN look like on cytology?

A

A bunch of dots present outside the chromosomes.

19
Q

What is homer wright pseudo rosettes?

A

Cellular formations making a complete circle. Can be seen in neuroblastomas.

20
Q

What labs can diagnose neuroblastoma?

A

Catecholamines in the blood (not as helpful) or catecholamine metabolites in the urine (VMA and HMA).

21
Q

What are some clinical presentations of neuroblastomas?

A

“Blueberry muffin rash”, large abdominal rash, periorbital bruising.

22
Q

What does neuroblastoma look like grossly?

A

Heterogenous appearing tumor sitting on the associated organ (kidney or spine). May see hemorrhage or necrosis.

23
Q

What does neuroblastoma look like on histology?

A

Small round blue cells and pseudrosettes.

24
Q

Describe retinoblastoma.

A

Malignant tumor that may be familial or sporadic that appears as a nodular mass in the posterior retina.

25
What is associated with familial retinoblastoma?
Osteosarcoma.
26
What is the RB1 gene?
A tumor suppressor gene. Mutation and deletion may lead to retinoblastoma.
27
What are Flexner wintersteiner rosettes?
Arrangements of cells with a true lumen that are often seen in retinoblastoma.
28
What is the white eye reflex?
Light shining on the eye reveals a white-ish background to the pupil that indicates retinoblastoma.
29
What is seen on histology in retinoblastoma?
Small round blue cells and wintersteiner rosettes.
30
What is a Wilm’s tumor?
Malignant pediatric tumor that impacts the kidney, aka nephroblastoma.
31
What are clinical signs of Wilm’s tumor?
Abdominal mass, aniridia, hematuria.
32
What are histologic features of Wilms tumors?
Triphasic histology (immature cells, some normal kidney features, and stroma), anaplasia (ugly af and mitotic figures), and nephrogenic rests.
33
What are nephrogenic rests?
A sort of precursor to Wilms tumor; an indicator that the other kidney may be at risk for Wilms tumor.
34
What is the gross pathology of Wilms tumor?
Bulky and pushing the borders of surrounding organ. Whiter as opposed to hemorrhagic.
35
What is WAGR syndrome associated with?
Wilms tumors, aniridia (lack of iris), genital anomalies, cognitive issues.
36
What gene is WAGR syndrome associated with?
Deletion on WT1.
37
What is denys-drash syndrome associated with?
Genital and renal tumors (Wilm’s tumors).
38
What gene is denys-drash associated with?
Negative mutation in WT1.
39
What is Beckwith Wiedemann associated with?
Big organs, hemihypertrophy, Wilm’s tumors.
40
What gene is Beckwith Wiedemann associated with?
WT2. Also associated with IGF2.
41
what are these, and what are they associated with
homer-wright pseudorosettes seen in neuroblastoma
42
what are these, and what are they associated with
flexner wintersteinder rosettes seen in retinoblastoma