1.Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA
Several histologic variants of CCSK are recognized. The most common variant is the myxoid CCSK. This histology features diffuse accumulation of mucopolysaccharide matrix material between tumor cells sometimes creating a cystic appearance. The sclerosing variant of CCSK is characterized by prominent collagen bundles that may isolate single or small groups of tumor cells in a dense matrix that may become hyalinized. The cellular pattern of CCSK is characterized by less extracellular matrix material between cells with overlapping of nuclei, a feature that may lead to confusion with a blastemal predominant Wilms tumor or primitive neuroectodermal tumor. Mitotic activity is usually increased in this variant. The epithelioid CCSK variant may be confused with nephroblastoma due to condensation of tumor cell cords. The palisading pattern is described as having spindle cell nuclei in parallel linear arrays alternating with nuclear free zones, a feature that resembles Verocay bodies of schwannomas. The spindle cell and storiform patterns are relatively uncommon. Anaplasia is a rare finding in CCSK (3% of cases), and is characterized by the presence of enlarged, hyperchromatic polypoid nuclei with multipolar mitotic figures. The nuclear accumulation of p53 in anaplastic tumors is thought to represent evidence of p53 gene mutation, a finding that has been well-documented in anaplastic Wilms tumors. The frequency of different CCSK variants is listed below:
Immunohistochemistry is rarely informative in CCSK. Immunoreactivity for the intermediate filament vimentin is usually present, however, reactivity with most other proteins including epithelial markers are negative.
Like other renal tumors of childhood, CCSK is staged by the National Wilms Tumor Study staging scheme as follows:
Stage I (25% of CCSK): For stage I tumors, 1 or more of the following criteria must be met:
Stage II (37% of CCSK): For Stage II tumors, 1 or more of the following criteria must be met:
Stage III (34% of CCSK): For Stage III tumors, 1 or more of the following criteria must be met:
Stage IV (4% of CCSK): defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region.
Stage V (not yet reported for CCSK): defined as bilateral renal involvement at time of initial diagnosis.
Of five patients reviewed at this institution, karyotypes were available for four of these. One patient had a clonal balanced translocation 10;17 and an interstitial deletion of the long arm of chromosome 14 as follows: 46, XY, t(10;17)(q22;p13)del(14)(q24.1q31.1).Three other patients had normal karyotypes. Fluorescent in-situ hybridization using a p53 probe was employed on the same cells harboring the clonal translocation above. This study documented the presence of two p53 signals on chromosome 17 indicating the absence of deletion or translocation of the TP53 tumor suppressor gene.
Noel A Brownlee ; Patrick Koty ; A Julian Garvin ; Mark J Pettenati
Kidney: Clear cell sarcoma with t(10;17)(q22;p13) YWHAE/NUTM2E
Atlas Genet Cytogenet Oncol Haematol. 2005-08-01
Online version: http://atlasgeneticsoncology.org/solid-tumor/5412/kidney-clear-cell-sarcoma-with-t(10;17)(q22;p13)-ywhae-nutm2e