Onkozytom der Niere
Renal oncocytomas are relatively benign renal tumors. The main clinical importance of this lesion is the difficulty in pre-operatively distinguishing it from renal cell carcinomas, as epidemiology, presentation, imaging and even histology can be very similar.
Epidemiology
Renal oncocytomas account for approximately 5% of resected primary adult epithelial renal neoplasms. They typically present in the 6 to 7 decades with a peak incidence at 55 years of age. There is 2:1 male predilection . Unfortunately, these demographics are similar to renal cell carcinoma.
Clinical presentation
Up to three-quarters of patients with a renal oncocytoma are asymptomatic. In cases where the mass is large, then a flank or abdominal mass may be the presenting complaint. Occasionally hypertension, hematuria or pain may be the presenting complaint .
Pathology
Macroscopic appearance
Macroscopically oncocytomas are tan in color, similar to the renal cortex, or darker brown (mahogany brown). A rim of compressed normal renal parenchyma is sometimes seen, forming a pseudo capsule . Macroscopic hemorrhage is seen in a minority of patients (~20%) .
Microscopic appearance
Oncocytomas are believed to originate from intercalated tubular cells of the collecting tubules and are composed of large, swollen eosinophilic cells of protuberant mitochondrial components . A biopsy is usually unhelpful in distinguishing an oncocytoma from a renal cell carcinoma, as the latter may also have oncocytic elements . Necrosis is usually absent .
Associations
Radiographic features
Unfortunately, the imaging appearance of oncocytomas is difficult to distinguish from renal cell carcinoma, and as such, they are usually resected . They appear as sharply demarcated lesions of variable size, but often large at presentation. A potentially helpful sign is the presence of a sharp central stellate scar, which although characteristic of oncocytomas, is only seen in a third of cases . Additionally, renal cell carcinomas may also demonstrate a central scar .
The only reliable feature is evidence of metastasis or aggressive infiltration into adjacent structures, in which case the diagnosis of renal cell carcinoma can be safely made.
It is important to carefully evaluate both kidneys, as up to 13% of patients have multiple oncocytomas (renal oncocytosis), and up to 32% have concurrent renal cell carcinomas .
Intravenous pyelogram
Oncocytomas appear as sharply demarcated, often large exophytic masses with enhancement during the nephrographic phase.
Ultrasound
Ultrasound demonstrates a well-circumscribed mass with echogenicity similar to the rest of the kidney. Occasionally a central scar may be visible .
CT
Tend be large well-demarcated tumors at presentation.
Non-contrast
- if less than 3 cm - homogenous attenuation
- if more than 3 cm - heterogeneous attenuation
- perinephric fat stranding may be present due to edema
- calcification may be present
Post contrast
- small tumors may enhance homogeneously, but usually enhancement is heterogeneous and the mass is larger
- central stellate non-enhancing scar is seen in approximately a third of cases
- renal vein thrombosis may be present, but tumor thrombus is absent
MRI
Typical signal characteristics include:
- T1: hypointense compared to renal cortex
- T2
- hyperintense compared to renal cortex
- may demonstrate hypointense central stellate scar
- T1 C+ (Gd): usually demonstrates homogeneous enhancement
Angiography
May demonstrate a spoke wheel pattern, of peripheral circumferential vessels penetrating towards the center of the lesion, which again, although characteristic of oncocytomas, may also be seen in renal cell carcinomas.
During the capillary phase, the tumor demonstrates a homogeneous blush, similar to normal renal parenchyma, lacking the 'bizarre' vascularity typically seen in RCCs . A lucent avascular rim may be seen due to the compressed pseudo capsule .
Microaneurysms have occasionally been reported .
Treatment and prognosis
Although renal oncocytomas are benign lesions and metastases are extremely uncommon , as they cannot be confidently pre-operatively distinguished from renal cell carcinomas, they are surgically resected. If the diagnosis is suspected pre-operatively then renal sparing surgery can be performed.
Differential diagnosis
General imaging differential considerations include
- renal cell carcinoma (RCC)
- the imaging appearances of RCCs and oncocytomas overlap to such a degree that confident pre-operative distinction is not possible
- metanephric neoplasms
- metanephric adenoma
- metanephric adenofibroma
- renal leiomyoma
Practical points
biopsy is usually unhelpful in distinguishing an oncocytoma from a renal cell carcinoma, as the latter may also have oncocytic elements
gross invasion of perirenal fat, tumoral extension into the renal vein, and regional lymphadenopathy on imaging would favor RCC instead of oncocytoma
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