Cystic nephroma explained

A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour (MEST) and renal epithelial stromal tumour (REST),^[1] is a type of rare benign kidney tumour.

Symptoms

Cystic nephromas are often asymptomatic. They are typically discovered on medical imaging incidentally (i.e. an incidentaloma).

Diagnosis

Cystic nephromas are diagnosed by biopsy or excision. It is important to correctly diagnose them as, radiologically, they may mimic the appearance of a renal cell carcinoma that is cystic.^[2]

Pathologic diagnosis

The characteristics of cystic nephromas are:

• Cysts lined by a simple epithelium with a hobnail morphology, i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts,
• Ovarian-like stroma that has a:
  • Spindle cell morphology, and has a
  • Basophilic cytoplasm.

Cystic nephromas have an immunostaining pattern like ovarian stroma; they are positive for:

• Estrogen receptor (ER),
• Progesterone receptor (PR) and
• CD10.

Differential diagnosis

• cystic partially differentiated nephroblastoma
• cystic standard nephroblastoma (cystic Wilm's tumor)
• cystic mesoblastic nephroma
• cystic renal cell carcinoma
• other renal cysts

Notes and References

1. Turbiner J, Amin MB, Humphrey PA, Srigley JR, De Leval L, Radhakrishnan A, Oliva E . Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term . The American Journal of Surgical Pathology . 31 . 4 . 489–500 . April 2007 . 17414095 . 10.1097/PAS.0b013e31802bdd56 .
2. Web site: Small JE, Jinnteh T, Hong X, Tuncali K, Seltzer SE, Ros PR . 11 August 2003 . Cystic Nephroma. . BrighamRAD Teaching Case Database. . https://archive.today/20041024000225/http://brighamrad.harvard.edu/Cases/bwh/hcache/385/full.html . 2004-10-24 .