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Case 17

Multilocular cystic nephroma

CT in an unusual cause of haematuria

This 44 year old patient was found to have microscopic haematuria at a routine examination. Ultrasound had demonstrated an abnormality of the left kidney, and so CT was performed.

Findings

There is a 4.5 cm well defined mass arising from the upper pole of the left kidney. This mass is lobulated, and consists of multiple water density cysts, separated by enhancing septa. No significant solid component is seen.

Diagnosis

Multilocular cystic nephroma (MLCN)

Differential

Cystic renal cell carcinoma

Discussion

MLCN (multiticular renal cyst, cystic nephroma) is a rare, non hereditary cystic neoplasm with a well defined capsule and multiple septa dividing epithelial lined cysts.

It has a biphasic age and sex distribution, being more common in males from 2 months to 2 years, and females in middle age (40-60 years). Children present with an asymptomatic mass, adults incidentally or with pain, haematuria or a mass. The haematuria often correlates with herniation of the mass into the renal pelvis, which is a well recognised feature. Haemorrhage and necrosis are rare. US and CT demonstrate a multioculated cystic structure unless the cysts are very small or filled with myxomatous material, when it can not be differentiated from any other solid renal mass. The septa may enhance on CT, the cysts do not. CT may demonstrate the intrapelvic or perirenal extension.

The tumour is generally benign but with variable growth rate and size (3 cm diameter to 18 x 34 cm, mean 9 cm dia). It is occasionally malignant with local recurrence and pulmonary spread. If MLCN is suspected pre-operatively, a partial nephrectomy may be appropiate for smaller lesions.

Further examples

These two images are taken from another case of MLCN. The first illustrates the mass, while the second shows the extension into the renal pelvis. A thin rim of contrast surrounds the mass, illustrating its intrapelvic location (black arrow).

This next example is of a much larger lesion, again a proven MLCN on histology.

References

Madewell JE et al. MLCN: A Radiographical Pathologic Correlation of 58 patients. Radiology 1983;146:309-21

Taveras JM & Ferruci JT. Radiology: Diagnosis - Imaging -Intervention (1986). Vol 4 Ch 117 p3-4.

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