Distinct features of teratoid Wilms tumor

Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy

“…This raises the question as to whether teratoid Wilms' tumor is generally recognized at the time of initial biopsy. This was not true for our case and 1 other case with lung metastasis [23]; the diagnosis was only made after nephrectomy. There are 2 possible explanations for this: the well-differentiated ("teratoid") component was missed by sampling error or the tumor differentiates after chemotherapy, and teratoid Wilms' tumor is a treatment effect rather than a separate biologic entity.…”

“…This was not related to a constitutional loss of WT1 expression because podocytes in the residual kidney showed positive nuclear staining. WT1 expression has only been studied in 3 other cases of teratoid Wilms' tumors; negative nuclear staining was reported in 1 case [20], whereas positive nuclear staining of the blastemal and nephrogenic tubular components was reported in 2 other cases [23], although the published image from this article does not confirm this. Additional studies are needed to confirm whether WT1 immunostaining might serve as a marker for this subtype of Wilms' tumor.…”

“…There are 2 possible explanations for this: the well-differentiated ("teratoid") component was missed by sampling error or the tumor differentiates after chemotherapy, and teratoid Wilms' tumor is a treatment effect rather than a separate biologic entity. A review of the 27 published cases [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] determined that 14 cases were diagnosed before chemotherapy; 5, on biopsy; 9, on nephrectomy; and 10 cases, after chemotherapy but did not have a biopsy before chemotherapy. For the remaining 3 cases, it could not be determined whether the diagnosis was before or after chemotherapy.…”

A comprehensive review of nephroblastoma with ureteric involvement

“…The mean patient presentation age in the present study was 47.3 months. While Ritchey and colleagues [9] described the SIOP staging profile in their study as stage I(10), II (18), III (14) and IV(3), the patients in the current study were staged as I(3), II (7), III(9), IV(10) and V(3). Advanced disease presentations in African patients for a variety of reasons are documented, including poor socioeconomic background and limited access to healthcare [20,21,24].…”

“…The pluripotency of nephroblastoma is exalted by its variable, but pathognomonic, epithelial, stromal and blastemal features [17]. Chemosensitivity and radiosensitivity characteristics have rendered it a highly curable malignancy [18]. With cure rates in excess of 90% in developed countries, nephroblastoma is viewed as one of the therapeutic successes of modern medicine [16].…”