Multilocular Cystic Renal Tumor in Children

Radiologic-Pathologic Correlation

Abstract Introduction and Historical Background Clinical Features

Pathologic Features Radiologic Features Differential Diagnosis Treatment and Prognosis

Summary Source Information References

Clinical Features

Multilocular cystic renal tumors generally are regarded as nonfamilial, are not associated with cystic changes in other organs, and are only sporadically reported in association with congenital abnormalities (16). A recent case report, however, described two cases of cystic nephroma and one case of pleuropulmonary blastoma in three siblings (19).

Because of their rarity, the true prevalence, age, and sex distributions of cystic nephroma and CPDN are difficult to determine. Eble (18) recently analyzed the data from almost 200 cases from four series. Although the data were not population based, but rather reflected consultation referral patterns in three instances (15,16,17) and a literature review in one (20), and thus may be subject to some selection bias, the data suggest a consistent demographic pattern. Two-thirds of multilocular cystic renal tumors occurred in children between 3 months and 2 years of age with a male predominance of 2:1. Of the tumors in the preceding group, almost 50% contained blastema and thus would properly be classified as CPDNs. Only 5% of the 200 cases occurred in patients 5 30 years of age. The remaining multilocular cystic renal tumors (approximately 30%) occurred in patients over 30 years of age, with an 8:1 female predominance; of this group, more than 80% proved to be cystic nephroma, and less than 20% were CPDN, with no sex predominance.

Most children with a multilocular cystic renal tumor come to attention when a painless abdominal mass is incidentally discovered by a caretaker or during routine physical examination. Progressive enlargement, which may be sudden and rapid, is well documented (16,21). Nonspecific signs and symptoms, including pain, hematuria, and urinary tract infection, are more common features in adults (16).

No serologic marker for these lesions has been identified, although a single case of CPDN with immunohistochemical and serologic demonstration of alpha-fetoprotein production has recently been reported (22).

Abstract Introduction and Historical Background Clinical Features

Pathologic Features Radiologic Features Differential Diagnosis Treatment and Prognosis

Summary Source Information References