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Host Defense > Path IX: neoplasia in infancy and childhood > Flashcards

Path IX: neoplasia in infancy and childhood Flashcards

1
Q

What is the typical presentation of cancer in adults?

A

epithelial cells that progress through sequential gene mutations within a single cell leading to a neoplastic clonal expansion of that cell.

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2
Q

What kinds of cells are typically invovled in pediatric tumors?

A

precursor cells, aka blastemal cells

in the organ or tissue invovled have the potential to differentiate into more than one cell line.

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3
Q

What is the children’s oncology group?

A

national organization that coordinates study protocols and research on the majority of pediatric tumors throughout the country

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4
Q

What is a common tumor of the kidney and what is the genetic mutation?

A

Wilms’ tumor (neuroblastoma)

WT1 mutation

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5
Q

What is a common adrenal tumor and genetic mutation?

A

neuroblastoma

N-myc amplification; ALK-1 mutation

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6
Q

What is a common pediatric CNS tumor?

A

medulloblastoma

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7
Q

What is Ewing’s sarcoma?

A

tumor of the soft tissue or bone caused by a t11;22 translocation

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8
Q

What is a tumor in the skeletal muscle called?

A

rhabdomyosarcoma

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9
Q

What are common signs of pediatric neoplasia, clinically?

A

failure to thrive, palpable mass on exam, fever, bone pain if metastatic

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10
Q

What are specific clinical signs of a renal tumor?

A

hematuria, oliguria, palpable flank mass

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11
Q

What are some clinical signs of an adrenal tumor?

A

hypertension if hormone secreting, hematuria if adrenal tumor invades kidney, palpable flank mass

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12
Q

What is a common clinical sign of hepatoblastoma?

A

elevated liver function tests

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13
Q

What is a common clinical sign of osteosarcoma?

A

pathologic fracture

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14
Q

What pediatric conditions might you see with a chest radiograph?

A

enlarged heart, lung infiltrates, pulmonary edema

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15
Q

What conditions would you see with an abdominal radiograph?

A

abnormal GI gas patterns, unusual calcifications and, with contrast, abnormal renal voiding patterns

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16
Q

What could you use an abdominal CT scan for?

A

defining intra-abdominal tumors: see if it involves kidney, adrneal gland, liver. Also tells you about consistency of the tumor.

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17
Q

When would MRI be particularly useful?

A

defining tissue planes btw soft tissue and bone as in osteosarcoma and Ewings sarcoma.

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18
Q

What is the origin of the neoplastic cell ion a neuroblastic tumor?

A

neuroblastic cells of the sympathetic autonomic system in either the adrenal medulla or sympathetic ganglia of the retroperitoneum

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19
Q

What is anew mutation recently found in neuroblastam and why is it important?

A

ALK1 mutation

may allow the addition of anti-tyrosine kinase chemocherapy

20
Q

How are neuroblastoma tumors divided histologically?

A

stroma-rich vs. stroma poor.
stroma rich is schwannian stroma- ganglioneuroblastoma and ganglioneuroma; stroma poor is neuropil pericellular matrix- undifferentiated, poorly differentiated cells

21
Q

What are six factors that make for an unfavorable histology for a neuroblastoma?

A

kid is older than 5, has a high MKI, diploid DNA ploidy, 1p deletion, 17q gain, or N-myc amplification

22
Q

What is wilms’ tumor

A

kidney tumor of ppl under the age of 10.

triphasic: blastemal, epithelial, and stromal/mesenchymal elements.

23
Q

What makes a Wilms’ tumor have unfavorable histology (3 features)

A
  1. Nuclei 3X the size of their neighbors
  2. hyperchromasia (incr. intensity of nuclear staining
  3. abnormal mitoses (multipolar)
24
Q

What is heterologous differentiation?

A

areas that have differentiated from blastemal cells but are not native to the kidney (ie. skeletal muscle)

25
Q

Ewings sarcoma/Askin tumor/primitive neuroectodermal tumor: what is the translocation common to them all?

A

TET family gene and an ETS-related gene translocation.

EWS/FLI1 translocation

26
Q

What are important markers for the diagnosis of ES/PNET?

A

IHC:CD99- high sensitivity but low specificity
FISH: look for the translocation
histochemistry: PAS with and without diastase. ES/PNET cells have PAS positiveglycogen that dissolves when pretreated with diastase
EM: can elucidate intracytoplasmic organelles

27
Q

What is osteosarcoma? Who is affected, where, and how does it present?

A

malignant neoplasm of osteoblasts that occurs during adolescence at sites of growing bones in the metaphysis of long bones near jts like the knee and hip. presents with pain and pathologic fracture
destroys cortical bone with periosteal reaction: codman triangle

28
Q

What is a histologic feature required for diagnosis of osteosarcoma?

A

osteoid production by neoplastic osteoblasts

29
Q

What are the four types of rhabdomyosarcoma? What kind of tumor is it?

A 
soft tissue tumor
embryonal RMS
botryoid
spindle cell
alveolar
30
Q

What is embryonal rhabdomyosarcoma? type, prognosis, sites, key cells

A

varied type
intermediate prognosis
various sites
contains strap cells- immature muscle cell called a rhabdomyoblast

31
Q

What is Botryoid RMS? type, prognosis, body location

A

subset of embryonal RMS
good prognosis
occurs in mucosal surfaces, esp. in the GU region or head and neck region.

32
Q

Spindle cell RMS: prognosis

A

best prognosis of RMS

33
Q

What is alveolar RMS? prognosis? translocation?

A

worst prognosis

translocation btw FOXO1a and PAX3 or PAX7.

34
Q

What are small round blue cell tumors of childhood? What distinguishes them?

A

poorly differentiated tumors occuring in viscera and soft tissue
distinguished by site, morphology, immunhohistochemical staining, genetic aberrations

35
Q

Where might you see a teratoma?

A

head and neck, sacrococcygeal, mediastinum

36
Q

What is an important histological feature of neuroblastoma?

A

homer-ritght rosettes (neuropil in the center)

37
Q

What is neuroblastoma?

A

arises from neural crest cells. adrenal is from the adrenal medulla and extra-adrenal is from the sympathetic ganglion

38
Q

What are clinical features of neuroblastoma?

A

most common tumor of embryonic origin. may present with metastatic disease, commonly to regional lymph nodes.

39
Q

What are the three neuroblastoma tumors?

A

neuroblastoma, ganglioneuroblastoma, ganglioneuroma. neuroblastoma is the least mature

40
Q

4 microscopic features of neuroblastoma:

A
  1. composed of neuroblasts: primitive small round cells with hyperchromatic nuclei
  2. formation of neuropil= extracellular eosinophilc fibrillary matrix
  3. intermixed with larger immature cells differentiating in the direction of ganglion cells
  4. homer-wright rosettes (central core of fibrillary material)
41
Q

What is the mitosis-karyorrhexis index for neuroblastoma?

A

number of karyorrhectic cells and mitotic figures based on 5000 cells in random fields

42
Q

What are some gross features of a Wilms tumor?

A

usually unilateral, encapsulated, well-circumscribed. cut surface is pale tan to light grey. hemorrhage, necrosis and cysts often seen

43
Q

Describe the metanephric blastema of a wilms tumor.

A

most primitive cell type- densely packed small round oval cells with scanty cytoplasm

44
Q

Describe the epithelial compoent of a wilms tumor

A

ranges from true rosettes to embryonic tumular structures

45
Q

describe the stromal compentn of a wilms tumor

A

loose, immature spindle cells resembling fibroblasts that may differentiate toward skeletal muscle

46
Q

What are three syndromes associated with Wilms tumors?

A 
beckwith wiedemann (WT2 deletion)
WAGRO (wilms aniridia, gu anomalies, retardation, obesity); denys drash (WT1 point mutation)
47
Q

what are the most common sites for rhabdomyosarcoma?

A

head and neck, GU, extremities

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