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Oncology I EMQ 1

Created: 5/8/2007
Updated: 11/1/2008

Hereditary renal cancer

A VHL syndrome
B Birt-Hogg-Dube
C Short arm of chromosome 7
D Hereditary renal cell carcinoma syndrome
E Hypoxia-inducible factor
F X-linked
G Short arm of chromosome 3
H Hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC)
I Fumarate hydratase
J Hereditary papillary renal carcinoma syndrome(HPRC)

1 Associated with type 2 papillary renal cell carcinoma
2 Associated with loss of 2 copies of a tumour suppressor gene
3 Location of the VHL gene
4 Associated with chromophobe RCC
5 The causative gene is the proto-oncogene c-MET
6 Inhibited by the VHL gene product


A Adenocarcinoma
B Squamous cell carcinoma
C Papillary carcinoma
D Carcinoma in situ
E Sarcoma
F Oncocytoma
G Conventional
H Chromophobe
I Transitional cell carcinoma
I  Medullary cell
J Angiomyolipoma

7 Arises from calyceal epithelium 
8 Associated with sickle-cell
9 Do not require follow-up 
10 Wunderlich's syndrome occurs in up to 10% of cases
11 80% occur sporadically 
12 Have a mahogony appearance and a spoke-wheel appearance on CT
13 The most common histological type of RCC


A Thiotepa
B Sorafenib
C Mitomycin
D Intravesical Bacille-Calmette-Guerin
E Sunitinib
F Doxorubicin
G Temsirolimus
H Methotrexate
I Taxanes

14 Interfere with the mitotic spindle
15 Cross-linking agent which inhibits DNA synthesis
16 Local immune-stimulant
17 Effective in the M-phase of the cell cycle
18 Has shown benefit versus placebo after failed prior immunotherapy in metastatic RCC
19 Antimetabolite
20 Has shown benefit over interferon as a first line agent in metastatic RCC


6E The VHL gene product destabilises HIF-1a and HIF-2a which in turn prevent overexpression of VEGF
Click here to read our article on the genetic basis of RCC

7I Medullary RCC's are thought to arise from calyceal epithelium
8I Medullary RCC's are associated with sickle-cell and carry a poor prognosis
9F Straight forward oncocytomas require no follow-up
10J Life-threatening retroperitoneal haemorrhage occurs in up to 10% of patients with AML's , and is known as Wunderlich's syndrome
11J 80% of AML's occur sporadically and 20% occur in association with tuberous sclerosis
12F Oncocytoma's characteristically have a brown/tan appearance and a spoke-wheel appearance on CT due to their central stellate scar
13G Conventional RCC includes clear-cell and granular-cell and is the most common histological subtype

14I Taxanes are spindle-tubule inhibitors and interfere with the mitotic spindle
15C Mitomycin is an antibiotic chemotherapeutic agent which is a cross-linking agent and inhibits DNA synthesis
16D The mechanism of action of intravesical BCG is unclear but it appears to be an immune stimulant, activating dendritic cells and upregulating cytokines in the bladder wall
17I As taxanes interfere with mitosis they are active in the M-phase
18B This was demonstrated by the Target Study NEJM 2007
19H Antimetabolites include methotrexate and gemcitibine
20E This was demonstrated by Motzer's phase III trial in NEJM 2007

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