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House AMP > WILM’S TUMOUR > Flashcards

WILM’S TUMOUR Flashcards

1
Q

Nephroblastoma

(Benign or Malignant?) __________ tumour

Occurs in (children or adults?)

A

Malignant

embryonal

children

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2
Q

Nephroblastoma

Does not occur in adults

T/F

A

F

Occurs in children, though rare forms in adult

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3
Q

Nephroblastoma

Origin : __________ __________ tissue (blastema)
Resembles histology of __________________

A

Nephrogenic embryonal

developing kidney

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4
Q

Nephroblastoma

Triphasic tumour:
___________
___________ (undifferentiated)
___________ components

A

Epithelial
Blastemal (undifferentiated)
Stromal components

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5
Q

Nephroblastoma: Epidemiology

Peak age: _____years

95% before ____years

A

2-5years

10years

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6
Q

Nephroblastoma: Epidemiology

Most common in _________
Least common in _____________
Slight female preponderance

A

Africans

East Asians

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7
Q

Nephroblastoma: Epidemiology

5 to 10% involve (one or both?) kidney(s)

Bilateral tumours associated with _________ mutations

10- 15% associated with syndromes and congenital anomalies

_____% are familial

A

both kidneys

germline

1-2%

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8
Q

Nephrblastoma: AETIOLOGY

Thought to develop from _______ tissue/ ___________

A

metanephric

nephrogenic rests

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9
Q

nephrogenic rests are ????

A

persistent clusters/groups of embryonal cell’s

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10
Q

Nephroblastoma: AETIOLOGY

Genetic alterations affecting embryonal development of genitourinary tract

_______ gene ___________

_______ gene

A

WT1

inactivation

WT2

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11
Q

_____ gene is required for normal gonadal and renal development

A

WT-1

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12
Q

Nephroblastoma: AETIOLOGY

Usually __________

1-2% _________

10-15% associated with syndromes: ______, __________ syndrome, Beckwith Wiedemann

A

sporadic

familial

WAGR; Denys-Drash

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13
Q

WAGR syndrome:

Full meaning ?

A

Wilm’s tumour, aniridia, genitourinary abnormalities, mental disability (retardation)

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14
Q

WAGR Syndrome

Lifetime risk of developing Wilm’s tumour is ______% , due to _______________ of _______ (WT-1gene)

A

33

Germline deletion

11p13

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15
Q

WT-1 gene = ____(p or q?) ——

A

11

p

13

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16
Q

Denys Drash Syndrome:

Characterized by ______________ and ______________ leading to renal failure

A

Male pseudohermaphroditism

Early-onset nephropathy

17
Q

Denys Drash Syndrome: ____% risk of Wilm’s tumor

Due to ___________ mutation in WT-1gene

A

90

Missense

18
Q

Nephroblastoma: AETIOLOGY

Beckwith Weidermann Syndrome (BWS):
Enlargement of body organs (organomegaly), __________ , __________,___________ , and abnormal _____ cells in the ___________ ( _______________)

A

macroglossia

hemihypertrophy

omphalocele

large; adrenal cortex; adrenal cytomegalovirus

19
Q

Nephroblastoma: AETIOLOGY

WT-2 locus (11p15.5)
Abnormality results from genomic imprinting (___________________ of one of the parental alleles (paternal or maternal?) )

A

transcriptional silencing

maternal

20
Q

Nephroblastoma: AETIOLOGY

Molecular changes associated with sporadic tumours still under investigation.

Genes implicated include ______,_______,_________

Tumours with ______ mutation have a poor prognosis

A

DICER1, DROSHA, DGCR8

TP53

21
Q

Nephroblastoma Tumours with TP53 mutation have a (poor or good?) prognosis

A

Poor

22
Q

Pathogenesis of Nephroblastoma???

A

Not clear how nephrogenic rests progress to tumour

23
Q

Wilm’s Tumor: Morphology- Gross

(Small or Large?)

(solitary or clustered ?)

(well or poorly?) -circumscribed mass

A

Large

Solitary

Well

24
Q

Wilm’s Tumor: Morphology- Gross

10% are ___lateral or ________ at the time of diagnosis

A

bi

multicentric

25
Q

Wilm’s Tumor: Morphology- Gross

(Soft or Hard?) , ______ to ________ color with foci of ____ formation, __________ and __________

A

Soft

grey to light brown

cystic; Haemorrhage; necrosis

26
Q

Wilm’s Tumor: Morphology- Microscopy

_______,__________, and __________ component in varying proportions

About 5% show __________ - presence of cells with large, hyperchromatic, pleomorphic nuclei and abnormal mitoses

A

Stromal, epithelial and blastemal

anaplasia

27
Q

Wilm’s Tumor: Morphology- Microscopy

Attempts to recapitulate different stages of nephrogenesis

Blastemal- sheets of _______ cells

Epithelial- ————-,__________

Stromal- ________,_________

A

small blue

abortive tubules, glomeruli

fibroblastic, myxoid

28
Q

Wilm’s tumor : Morphology- Microscopy

______________ are putative precursor lesions of Wilms tumors and are seen in the renal parenchyma adjacent to approximately 25% to 40% of _______ tumors
Nearly 100% in _______ Wilms tumors

A

Nephrogenic rests

unilateral

bilateral

29
Q

Wilm’s tumor : Morphology- Microscopy

It is important to document the presence of nephrogenic rests in the resected specimen

T/F

With reason

A

T

These patients are at an increased risk of developing Wilms tumors in the contralateral kidney

30
Q

Staging and Grading of Wilm’s tumors

________ or ________

A

SIOP or COG

31
Q

CLINICAL FEATURES: Wilm’s tumors

(Small or Large?) _______ mass that may be unilateral or, when very large, extend across the ——— and down into the ______. ___________,

______ in the abdomen after a traumatic incident, __________ and _________ are other patterns of presentation.

A

Large ; abdominal

midline; pelvis; Hematuria

Pain; intestinal obstruction, and hypertension

32
Q

CLINICAL FEATURES: Wilm’s tumors

In a significant number of these patients, _________ metastases are present at the time of primary diagnosis.

A

pulmonary

33
Q

Wilm’s Tumor: TREATMENT

________,__________,___________

A

Nephrectomy
Chemotherapy
Radiotherapy

34
Q

Wilm’s Tumor: PROGNOSIS

________________ is perhaps the most important determinant of adverse prognosis as it confers increased risk of recurrence and death

Need for accurate identification of this histologic feature.

A

Anaplastic histology

35
Q

Wilm’s Tumor: Prognosis

The presence of anaplasia correlates with the presence of _______ mutations and the emergence of _______________________

A

TP53

resistance to chemotherapy.

36
Q

Wilm’s Tumor: Prognosis

P53 elicits —————- signals in response to DNA damage

The loss of p53 function might explain the relative ____________________________ to cytotoxic chemotherapy

A

pro-apoptotic

unresponsiveness of anaplastic cells

37
Q

Wilm’s Tumor: Prognosis

Individuals with Wilms tumor have an increased risk of developing second primary tumours, including ________ and soft tissue ________ , ________ and ________, and ________ cancers.

Although some of these neoplasms result from the presence of a germline mutation in a cancer predisposition gene, others are a consequence of therapy, most commonly radiation administered to the cancer field

A

bone and soft tissue sarcomas, leukemia and lymphomas, and breast cancers.

House AMP (65 decks)

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