The ONLY primary renal tumour which expresses HMB-45 is:
Answer A
AML is the only primary renal tumour that expresses HMB 45. If a primary tumour stains positive for HMB 45, it is an AML.
The MOST common form of RCC in patients in end-stage renal disease and in patients on dialysis is:
Answer B
Acquired cystic kidney disease [ACKD] and a higher incidence of RCC are typical features of ESKD (end-stage kidney disease). RCCs of native end-stage kidneys are found in about 4% of patients. The lifetime risk of developing RCCs is at least 10 times higher than in the general population. Compared with sporadic RCCs, ACKDs generally are multicentric and bilateral, found in younger patients (mostly male) and are less aggressive. The relatively indolent outcome of tumours in ESKD is due to the mode of diagnosis and a specific ACKD related molecular pathway still to be determined. Although the histological spectrum of ACKD tumours is similar to that in sporadic RCC, the predominant form is papillary RCC.
All of the following features are TRUE about metastatic RCC, except:
In clinical practice, 20% of patients present with metastatic disease. When a metastatic disease is discovered, a solitary metastasis is present only in 1% of cases. Metastases occur by lymphatic and hematogenous spread with equal frequency. Distant metastasis is present in >50% of patients with regional lymph node metastasis. The most common site of metastasis (most to least) is lung, bone, regional lymph nodes, liver, adrenal gland, contralateral kidney and brain. The most common site of bone metastasis is spine. Risk of metastasis on presentation for 3–4 cm, 4–7 cm, 7–10 cm and 10–15 cm renal tumours is 7%, 16%, 30% and 41%, respectively. If metastasis develops after nephrectomy for an M0 renal cancer, they usually occur within one year of surgery
Which of the following is TRUE about tuberous sclerosis?
Answer E
Tuberous sclerosis (TS) is an autosomal dominant disease caused by the mutation of TSC1 gene (chromosome 9) and TSC2 gene (chromosome 16). The classic triad of tuberous sclerosis (adenoma sebaceum, seizures and mental retardation) is seen in only 30% of the patients. Adenoma sebaceum are pink- or red-coloured papules usually present on nasolabial folds. They usually develop between age 4 and puberty. Urologic manifestations – renal cysts (usually develop in childhood), AML (usually B/L and multifocal), RCC (in 2% of patients).
The following risk factors are included in BOTH MSKCC risk models and IMDC risk models, except:
Answer C
MSKCC and IMDC – The MSKCC (Motzer) criteria was developed during the cytokine era and the International Metastatic Renal Cancer Database Consortium (IMDC) risk model was established and validated to yield an accurate prognosis for patients treated in the era of targeted therapy. In IMDC risk model, neutrophilia and thrombocytosis have been added to the list of MSKCC risk factors, while LDH has been removed.
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