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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Rhabdoid variant of clear cell renal cell carcinoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 18 July 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Typically children, 5% adults (mean age 62 years), usually in transition with conventional clear cell renal cell carcinoma (Am J Surg Pathol 2000;24:1329)
● A distinct histologic entity in children, but not in adults (merely descriptive)
● Mean age 62 years (range 33-84 years), 2/3 men
● Rhabdoid cells make up 5-90% of tumor cells

Clinical description
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● Abdominal mass, rarely hematuria or flank pain
● Usually high stage and high grade with Fuhrman grade 3 or 4 nuclei in non-rhabdoid component and aggressive behavior similar to sarcomatoid carcinoma; median survival only 8 months (Arch Pathol Lab Med 2007;131:102)
● Often lymph node or pulmonary metastasis (J Urol 2011;186:675)

Case reports
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● 47 year old woman (Can Urol Assoc J 2008;2:631)
● 82 year old woman (Arch Pathol Lab Med 2004;128:109)
● In mixed epithelial and stromal tumor (Hum Pathol 2007;38:1432)

Gross description
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● Rhabdoid component is white, firm, homogeneous

Micro description
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● Sheets/clusters of variably cohesive epithelioid cells with large paranuclear intracytoplasmic hyaline globules, vesicular and often eccentric nuclei, prominent nucleoli
● Rhabdoid foci always high grade, represents 5-50% of tumor volume
● Tumor necrosis common and sometimes extensive
● Usually no prominent vasculature; non-rhabdoid component also has high nuclear grade

Micro images
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Various images


Figure 1: kidney has white circumscribed nodules
Figure 2: rhabdoid component is distinct from clear cell component
Figure 3A: eosinophilic cytoplasm has intracytoplasmic globulins
Figure 3B: leukophagocytosis present
Figure 4A: vimentin+ clear cells
Figure 4B: vimentin+ rhabdoid cells



p53 staining

Positive stains (rhabdoid component)
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● Vimentin (100%), NSE (79%), PAS (glycogen), pan cytokeratin (56%), EMA (47-80%), p53 (70% had at least 5% positive cells in rhabdoid areas), S100 (37%)
● Paranuclear globular staining is present for CK, EMA, vimentin

Negative stains (rhabdoid component)
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● Desmin, smooth muscle actin
● Also CK7, CK20, muscle specific actin, HMB45, GFAP (J Urol 2011;186:675)

Electron microscopy description
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● Inclusions composed of paranuclear whorled aggregates of intermediate filaments or condensed organelles, often with peripheral vacuolization (Histopathology 2002;41:538)

Additional references
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J Urol 2011;186:675

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Rhabdoid variant of clear cell renal cell carcinoma


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