Case Report: Cystic Partially Differentiated Nephroblastoma (Wilms Tumor)

Abstract

A case of a cystic partially differentiated nephroblastoma treated with combined chemotherapy after removal in a 2-month old newborn is reported. Cystic partially differentiated nephroblastoma is a newly described clinicopathologic entity, involving elements of a nephroblastoma and cystic disease, and it is believed to be a pathologic derivative of the metanephric blastema and not related to renal dysplasia. Cystic partially differentiated nephroblastoma is distinguished from multilocular cysts of the kidney by the presence of partially differentiated renal elements in the septa of cysts. The aggressive appearance of the cells led to the treatment of this tumor as potentially malignant.

Partial nephrectomy in a cystic partially differentiated nephroblastoma

“…A dilemma occurs in patients where relatively undifferentiated cellular elements are found within the cyst. To clarify whether these patients benefit from adjuvant therapy or not, Cas- strate that the vast majority of cases of CN, CPDN and cystic nephroblastoma show no evidence of tumor recurrence or metastasis despite the fact that no chemotherapy and radiotherapy had been administered [30][31][32][33][34][35].…”

Cystic partially differentiated nephroblastoma in an adult: an immunohistochemical, lectin histochemical and ultrastructural study

“…Because of its rarity, only scanty data are available regarding the nature and characteristics of both the epithelial and nonepithelial components included in CPDN. Almost all patients with CPDN reported to date have been less than 24 months old, 1 –7 and the few remaining cases have occurred in older children 7 . To our knowledge, no adult case of CPDN has ever been reported.…”

“…Cystic partially differentiated nephroblastoma (CPDN) is a rare solitary multicystic tumour of the kidney, usually occurring in early infancy 1 –6 . Brown described the first case of CPDN in 1975, 1 and thereafter, Joshi and Beckwith established the criteria for diagnosis of CPDN as a distinct entity from cystic nephroma 7 .…”