Back to Pediatric Renal Home Page

REVIEW OF PEDIATRIC RENAL NEOPLASMS
John Hicks
Texas Children's Hospital, Houston, TX

CLEAR CELL SARCOMA OF KIDNEY

INDEX

 

Clear Cell Sarcoma of Kidney: Epidemiology
Bone Metastasizing Renal Tumor of Childhood

Clinically Aggressive and Widespread Metastases
"Unfavorable Histology" NWTS

Extremely Rare in First 6 Months of Life

Peak Incidence in Second Year of Life
(No cases beyond third decade of life)

Gender Ratio 1.6M:1.0F

 
 

Clear Cell Sarcoma of Kidney: Pathology/Outcome
Median Size of Tumor = 520 grams
(Range = 126 to 3,000grams)

Unilateral and Unicentric Tumors
Cysts Common

Tumor-Kidney Junction Sharply Defined

Arise from Medullary Pyramids or Central Renal Parenchyma
(Blastemal Cap Cells, Metanephric Blastema, Mesenchyme
Renomedullary Interstitial Cells)

Immunocytochemistry: Negative for keratin, EMA, MSA, Desmin; Positive for Lectins, alpha-1-antichymotrypsin

Patterns

 Classic  Epithelioid  Spindled
 Sclerosing  Myxoid  Cystic
 Palisading  Pericytomatous  Pleomorphic (anaplastic)

 

Clear Cell Sarcoma of Kidney:  
Light microscopy (A) and Ultrastructure (B)
Clear cell sarcoma- lm Clear cell sarcoma - em
Figure 3A: Relatively bland cohesive  
cells with lightly eosinophilic to amphophilic cytoplasm form this tumor.  Frequent mitotic figures are common.		 Figure 3B: Tumor cells have a bland nuclei  
and sparse cellular organelles. 
 

   

Clear Cell Sarcoma of Kidney: Pathology/Outcome
Typical Morphology:
Cords and Columns of Polygonal Cells
Delicate Interlacing Fibrovascular Network
Amphophilic Cytoplasm
Infiltrates Adjacent Normal Kidney
Scattered Renal Tubules within Tumor
Cysts

Relatively Low Mitotic Rate
Diploid Tumors with Low Proliferative Fraction

Most No Cytogenetic Abnormality
One Case with t(10;17)

Metastatic Disease:
Bone: Initially 40 to 70% -->More Recently 17%
Nodes, Brain, Lung, Liver, Soft Tissue, Muscle, Testis,
Salivary Gland

Late Relapses (as long as 5 years)

Prognosis:
Mortality Decreased from 70% --> 30 to 40%
Median Survival 1.4 years
2 Year Survival 39 to 49%


INDEX
Nephroblastoma (Wilms tumor)
Congenital Mesoblastic Nephroma
Malignant Rhabdoid Tumor
Renal Cell Carcinoma
Angiomyolipoma
Ossifying Tumor of Infantile Kidney

Top of page

SUP Logo