Multilocular cystic renal tumour

Dr Jeremy Jones and A.Prof Frank Gaillard et al.

Multilocular cystic renal tumours (MCRT) are rare benign renal neoplasms occurring in a bimodal age distribution, involving young children and adults in the 4th and 5th decades.

For logical reasons, this article will discuss together the two ends of the spectrum of this disease, cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma, as they are indistinguishable from one another based on radiographic appearances and gross pathology. 

These tumours are rare and have a bimodal age and sex distribution:

  • paediatric (3 months to 4 years of age) - 75% male predilection
  • adult (typically in the 5th - 6th decades) - females are predominantly affected

In paediatric patients, a multilocular cystic nephroma usually present as an asymptomatic palpable abdominal mass felt by parents, with increasing abdominal girth. It may also present with haematuria or possibly urinary tract infection

Grossly, multilocular cystic renal tumours are typically unifocal multiloculated cystic masses surrounded by a thick fibrous capsule and compressed parenchyma 8. Calcification, haemorrhage, and necrosis are unusual. 

Microscopically, these tumours are lined by flat, cuboid, or hobnail cells 8, while the fibrous septa may contain mature tubules 3.

A familial association of cystic nephroma with the cystic type of pleuropulmonary blastoma related to DICER1 mutation has been described 7

Multilocular cystic renal tumours generally have the appearance of a multilocular cystic encapsulated mass on most modalities.

May present as a large abdominal mass displacing and effacing adjacent bowel loops.

  • multilocular cystic mass originating from kidney 4 
    • claw or beak-shape of adjacent renal parenchyma (claw sign) may help confirm a renal origin
  • cyst contents usually anechoic, but low-level echoes may be seen
  • septal vascularity can also be seen 8
  • multilocular cystic mass often herniates into the renal pelvis
  • variable septal enhancement
  • no nodular or solid enhancement 8 
  • +/- associated streakiness in perirenal fat 4

Signal characteristics include:

  • T1: variable signal, depending on the protein or blood products of the cysts
  • T2: hyperintense (cysts)
  • T1 C+ (Gd): septal enhancement may be seen

Radical or partial nephrectomy is usually done, with lymph node excision.

In an adult population, it is indistinguishable from multilocular clear cell renal cell carcinoma.

In a paediatric population, it is often difficult to distinguish cystic Wilms tumour from the multilocular cystic renal tumours:

  • solid nodular components in a tumour should arouse suspicion of Wilms tumour 9
  • MCRT tends to occur in younger children than Wilms tumours

Multicystic dysplastic kidney (MCDK) is a developmental anomaly in which the entire kidney is replaced by noncommunicating cysts of varying sizes. It is usually diagnosed prenatally or at birth, while multiloculated cystic renal tumours do not occur in the perinatal period 8. In addition, enhanced compressed renal parenchyma is seen to surround cystic nephroma on contrast-enhanced cross-sectional studies, while this tissue is absent in MCDK

Share article

Article information

rID: 4949
Synonyms or Alternate Spellings:
  • Multilocular cystic renal tumour
  • Renal cystic adenoma
  • Segmental polycystic kidney
  • Renal cystic hamartoma
  • Benign cystic nephroma
  • MLCN
  • Multilocular cystic nephroma (MLCN)
  • Multilocular cystic nephroma
  • Cystic nephroma
  • cystic partially differentiated nephroblastoma

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Figure 1: gross pathology
    Drag here to reorder.
  • Drag
    Figure 2: histology
    Drag here to reorder.
  • Drag
    Case 1: adult form
    Drag here to reorder.
  • Drag
    Case 2: paediatric form
    Drag here to reorder.
  • Updating… Please wait.

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.