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Repro > Tumors of Early Childhood > Flashcards

Tumors of Early Childhood Flashcards

1
Q

What is the most common death in children?

A

Accidents (40%) Cancer only accounts for 11%

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2
Q

What is the most common malignancy in children?

A

Leukemia

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3
Q

What is the biggest difference in type of tumors in the pediatric vs adult population?

A

Carcinoma (epithelial-derived tumors) are not uncommon in adults while they are VERY rare in children

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4
Q

What is a neuroblastoma?

A

Neuroendocrine tumor arising from any neural crest element of the sympathetic nervous system -2nd most common malignancy in childhood

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5
Q

What is the median age of diagnosis of a neuroblastoma?

A

22 months

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6
Q

What is the most common site of a neuroblastoma?

A

Adrenal glands

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7
Q

Two clinical presentations of a neuroblastoma

A

Often fever w/ (a) palpable abdominal mass- often of the adrenals (b) Elevated urine catecholamine metabolites 90% of neuroblastomas secrete catecholamines

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8
Q

Differentiate neuroblastomas from ganglioneuromas

A

Depends on the percentage of Schwannian development 50% Schwannian development = Ganglioneuroma

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9
Q

Describe the staging of neuroblastomas

A

Typical stages I-IV I- tumor confined to structure of origin III- LN IV- distant mets Special Stage IV-S = stage I or II tumor w/ remote disease in the liver, skin, or bon marrow -very good prognosis -must be

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10
Q

What is distinct about Stage IV-S

A

Stage 4-S is a distinct stage of neuroblastoma where the primary tumor is of stage I-II, but there is remote disease in the liver, skin, or bone marrow -very good prognosis despite distant mets -must be an infant (

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11
Q

Better prognosis for children over of under 1 year of age dx w/ neuroblastoma

A

Better prognosis for those

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12
Q

What gene is amplified in many cases of neuroblastoma?

A

N-myc -amplification of N-myc oncogene is a common finding in neuroblastoma and is associated w/ bad prognosis

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13
Q

What tumor contains pseudo-rosettes on histology?

A

25-30% of neuroblastomas show pseudo-rosettes

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14
Q

Describe the classic histological appearance of a neuroblastoma

A

Presence of neuropil (stromal background) within a small round blue cell tumor -neuropil = fine, fibrilliary, eosinophilic material

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15
Q

What is a Wilm’s tumor?

A

= Nephroblastoma -malignant neoplasm derived from nephrogenic blastema -most common renal tumor of childhood

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16
Q

Median age of diagnosis of Wilm’s tumors

A

3-4 yoa

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17
Q

What percent of Wilm’s tumors are syndromic? What gene is commonly mutated in syndromic Wilm’s tumors?

A

10% of Wilm’s tumors are syndromic, due to mutation in WT-1 gene -WAGR syndrome = deletion of WT1 -Denys-Drash syndrome = missense mutation in WT1 Beckwith-Wiedemann syndrome = WT2 disruption

18
Q

Which childhood tumor do you not want to biopsy?

A

Wilm’s tumor -don’t want to biopsy b/c any rupture of the encapsulation puts the patient at risk of hemorrhage age peritoneal dissemination -so pre-surgical biopsy is rarely (if ever) indicated b/c it will result in automatic upstaging of the tumor

19
Q

What is the most common presentation of a Wilm’s tumor?

A

Enlarged abdomen -usually due to mass

20
Q

What is the most important histological feature for predicting prognosis and response to treatment of a Wilm’s tumor?

A

Absence or presence of anaplasia Anaplasia defined by mitotic figures and nuclear enlargement

21
Q

Describe what it means to be a triphasic tumor?

A

Triphasic = comprises 3 elements, one from each of the categories (a) Blastema- cells that can grow/regenerate into organs or body parts (b) Mesenchyme- derivative of CT (c) Epithelium

22
Q

What is an Ewing/PNET (Primitive neuroectodermal tumor)

A

Malignant (cancerous) neural crest tumor, cells aren’t fully developed into neurons => called primitive

23
Q

Tumor that diffusely stains for CD-99 on immunohistochemistry

A

Ewing/PNET

24
Q

What is the genetic etiology of Ewing/PNET?

A

Translocations- most common is the EWS/FLI-1 fusion gene -EWS-FLI-1 fusion gene due to reciprocal translocation. Transcription factor that upregulates FLI-1 expression

25
Q

How is a Ewing/PNET tumor diagnosed?

A

Molecular testing for fusion gene -can use FISH to detect breaking apart of EWS gene

26
Q

Differentiate PNET from (a) Osteosarcoma (b) Desmoplastic small round blue tumor

A

Primitive neuroectodermal tumor (2nd most common bone cancer in adolescents) (a) Osteosarcoma = most common bone cancer in adolescents. Produces osteoid (bone matrix) from tumor cells (b) Desmoplastic small round blue tumor is primarily extraskeletal and will stain for WT-1

27
Q

What are rhabdomyosarcomas? How are they classified?

A

Sarcomas (cancer of CT) where cancer cells arise from skeletal muscle progenitors -classified histologically

28
Q

Which rhabdomyosarcoma has (a) best prognosis (b) worst prognosis

A

Rhabdomyosarcoma (a) best prognosis = botryoid (b) worst prognosis = alveolar intermediate = embryonal

29
Q

What is the most common subtype of rhabdomyosarcoma?

A

Embryonal subtype -intermediate prognosis

30
Q

What is the most common location of an embryonal rhabdomyosarcoma?

A

Orbit of the eye, nasal/oral cavity -most common 3-10 yoa

31
Q

Histological and immunofluorochemical appearance of embryonal ehabdomyosarcoma?

A

Spindle appearance histologically -immunofluorochemically: positive for myoD-1 and myogen (skeletal muscle markers)

32
Q

What is the gross appearance of botryoid rhabdomyosarcoma?

A

Bunch of grapes protruding from the vagina

33
Q

What is the histological appearance of botryoid rhabdomyosarcoma?

A

Cambium layer under the surface = hypercellular condensed layer of growth

34
Q

Which rhabdomyosarcoma is associated w/ PAX fusion gene?

A

Alveolar rhabdomyosarcoma (worst prognosis- 54% 5 yr survival)

35
Q

Which rhabdomyosarcomas stain positive for skeletal muscle markers?

A

Embryonal stain for myoD-1 and myogen but not diffusely Alveolar stain diffusely

36
Q

What is the most common intraocular tumor of children?

A

Retinoblastoma

37
Q

What percent of retinoblastomas are familial? Mutation in what gene?

A

60% sporadic, 40% are familial and associated w/ autosomal dominant inheritance of Rb gene

38
Q

What is leukocoria an indication of?

A

Leukocoria (abnormal white reflection from the retina of the eye) is the most common clinical presentation of retinoblastoma

39
Q
A

Cambium layer of botryoid rhabdomyosarcoma (grapes out of vagina) = layer immediately under the surface is a hypercellular condensed layers of cell growth

40
Q
A

Triphasic pattern of Wilms tumor (nephroblastoma)

41
Q
A

Small blue cell tumor w/ neuropil stroma in background = Neuoblastoma

42
Q

What tumors are most common w/in the first 5 years of life?

Which tumors are more common in the teens?

A

Early life: neuroblastoma, wilms tumor, Retinoblastoma, PNET

Later teen years: osteosarcoma,

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