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Kidney tumor

Other adult malignancies

Wilms’ tumor - adult


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 28 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare, prognosis considered poor in past, but now overall survival is 83% (J Clin Oncol 2004;22:4500)
● Reports showing worse overall survival compared to pediatric patients may be due to actual diagnosis of PNET/Ewing’s, clear cell sarcoma of soft parts or synovial sarcoma (Am J Surg Pathol 2000;24:1663), inadequate staging, undertreatment (Cancer 2012;118:2541), but see Can J Surg 2008;51:252 (prognosis is actually poorer in adults)

Epidemiology
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● Mean 32 years old, range 21-67 years (for patients greater than 18 years old)
Criteria for diagnosis: primary renal neoplasm with primitive blastematous spindle or round cell component, abortive or embryonal tubular or glomeruloid structures (i.e. at least biphasic morphology), no renal cell carcinoma or other histology, age 15 years+

Poor prognostic factors
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● Large size, high mitotic rate (Mod Pathol 1990;3:321)

Case reports
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● 37 year old woman (Journal of Clinical Medicine Research 2010;2:194)
● 48 year old man (Diagn Pathol 2006;1:46)
● 50 year old man with teratoid tumor (Pathol Int 2009;59:44))
● 54 year old man with intracaval and intracardiac extension (J Cancer 2011 Mar 2;2:132)
● 62 year old woman (Arch Pathol Lab Med 2003;127:245)

Gross description
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● Large (mean 12 cm), nodular, bulging, gray-white

Gross images
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Tumor untreated for 5 years

Micro description
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● Similar to pediatric Wilms’ with varying amounts of blastema, epithelium and stroma (triphasic)
● Many “monophasic Wilms’” are actually PNET/Ewing’s
● Anaplasia is unusual
● No persistent renal blastema

Micro images
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Tubular pattern may resemble pseudorosettes

       
Triphasic tumor

   
Immunostains


Figure 1: CT scan shows 15 cm tumor of right kidney
Figure 2: gray-yellow lobulated tumor
Figure 3a: large tumor cells exhibit molding
Figure 3b: focal marked pleomorphism
Figure 4a: focal abnormal mitotic figures
Figure 4b: keratin+ squamous epithelium
Figure 4c: focal glomeruloid structures


Positive stains
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● Cytokeratin, CD56; blastema is rarely CD99+

Negative stains
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● Vimentin, CD99

Molecular description
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● Associated with 11p15 (Wilms’ tumor suppressor gene product), isochromosome 7q
● Adult tumors may have increased genetic complexity compared to pediatric tumors, suggesting a distinct pathogenesis
(Virchows Arch 2011;459:547)

Differential diagnosis
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Ewing sarcoma/PNET: rosettes, no blastema, no epithelial structures, CD99+, FLI1+, 90% have EWS/FLI1 fusion product by RT PCR due to t(11;12)(q24/q22;q12)
Clear cell sarcoma of soft parts: nests or short fascicles of spindled or epithelioid cells with clear to granular eosinophilic cytoplasm separated by fibrous septa; S100+, HMB45+, keratin-, t(12;22)(q13;q12)
Sarcoma NOS: no epithelium (keratin-)
Rhabdoid tumor: rhabdoid type cells; loss of INI1

End of Kidney tumor > Other adult malignancies > Wilms’ tumor - adult


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