Bonsib_EU_EU Urology 17/03/2010 15:15 Page 36
Renal Cell Carcinoma
Figure 1: Typical Large Invasive Clear-cell Figure 2: Classic Histological Appearance of a Clear-cell
Renal Cell Carcinoma Renal Cell Carcinoma
There is massive main renal vein involvement (bottom centre) and peripheral peri-nephric This type of tumour is characterised by a nest of cells that have complete cytoplasmic
fat invasion (top, centre left). clearing invested by a delicate capillary plexus.
Figure 3: Electron Micrograph of a Clear-cell Figure 4: von Hipple-Lindau Disease
Renal Cell Carcinoma
Image shows the abundant black granular glycogen granules and large grey lipid (L) droplets Image shows a clear-cell-lined cyst (right) and a solid microscopic or ‘incipient’ clear-cell
that are extracted during processing of tissue for light microscopy. On the right is a tight renal cell carcinoma. Many similar lesions were present elsewhere in this kidney.
cluster of intracellular microvilli (arrow).
TSC, an autosomal dominant disease with dysgenic lesions in like cells resembling a high-grade carcinoma but with a distinctive
multiple organs, results from a mutation of TSC1 or TSC2 tumour immunophenotype. It stains for melanoma markers but not epithelial
suppressor genes that encode for hamartin and tuberin.
30–35
Renal markers. Approximately 30–50% behave in a malignant fashion.
77,78
involvement occurs in 50–80% of patients and consists of multiple
and bilateral angiomyolipomas, renal cysts and cystic renal disease Suspicion that a patient with CC-RCC has TSC should be followed up if
and malignant renal neoplasms. Two types of malignant renal additional lesions are also present. If a single AML is detected with a
neoplasm develop in approximately 3% of patients.
68–79
The first is CC-RCC, TSC should be considered, especially in a young patient. If two
CC-RCC histologically identical to the conventional CC-RCC but or more AMLs are identified, this should be construed as an atypical
lacking the 3p deletion and vHL gene mutations characteristic of form of TSC, regardless of the absence of extrarenal stigmata. Another
conventional CC-RCC, discussed below.
30,68–76
The second is an ‘red flag’ is the presence of multiple cysts. Patients with a TSC2
unusual variant of angiomyolipoma (AML) known as the epithelioid mutation may have an associated mutation of the PKD1 gene located
AML.
77–79
Epithelioid AML is composed of large eosinophilic ganglion- adjacent to the TSC2 gene on chromosome 16. Dual mutation is known
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