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Kidney tumor - adult malignancies

Other neoplasms adult or adult/child

Leiomyosarcoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 30 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare, <200 cases reported; 0.12% of all invasive renal malignancies

Epidemiology
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● Mean age 58 years, range 40-75 years; 70% women in one study
● Median overall survival is 25 months, 5 year overall survival is 25% (Can J Urol 2007;14:3435)
● Patients usually die of disease, particularly if intermediate or high grade tumors (Am J Surg Pathol 2004;28:178)

Case reports
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● 42 year old woman with tumor arising from blind end of a bifid renal pelvis (Yonsei Med J 2007;48:557)
● 55 year old woman (Patholog Res Int 2010;2010:652398)

Gross description
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● Mean 14 cm, usually circumscribed and encapsulated with firm and whorled cut surface
● Usually necrosis

Gross images
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Tumor is solid nodule that arose in musculature of renal vein


Tumor has large intrarenal component


Arising from bifid pelvis


Necrosis and compressed rim of kidney parenchyma


Necrosis, hemorrhage and focal dilated kidney

Micro description
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● Alternating fascicles of spindle cells with eosinophilic cytoplasm and blunt ended, nontapering nuclei
● Usually focal necrosis (<50% of specimen), often myxoid areas
● Mean 10 mitotic figures/10 HPF but wide range (3-43 mitotic figures/10 HPF)
● Minimal inflammation; occasional vascular invasion
● Usually no epithelioid cells or marked pleomorphism

Micro images
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Intersecting fascicles of smooth muscle cells


Various images


SMA+, keratin-

Positive stains
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● Smooth muscle actin, desmin, calponin, h-caldesmon

Negative stains
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● EMA (usually), cytokeratin, S100, HMB45

Differential diagnosis
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Angiomyolipoma: triphasic, may have degenerative changes but no marked atypia, no mitotic activity, positive for melanocytic markers
Leiomyoma: not infiltrative, no atypia, no mitotic figures, no necrosis
Sarcomatoid renal cell carcinoma: more pleomorphic, no alternating fascicles, usually no smooth muscle morphology, usually keratin+ and muscle marker negative

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Leiomyosarcoma


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