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Kidney tumor - cysts, children, adult benign

Benign (usually) adult tumors

Inflammatory myofibroblastic tumor


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 27 March 2012, last major update January 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare spindle cell tumor in kidney with an inflammatory component

Epidemiology
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● Rare; presents at a median age of 57 years, range 2176 years, no gender preference
● In GU, usually occurs in bladder but also reported in kidney, renal pelvis, ureter

Clinical features
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● Patients may present with hematuria, flank pain or hydronephrosis

Case reports
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● 37 year old man and 54 year old woman (Arch Pathol Lab Med 2000;124:1209)
● 56 year old man (World J Surg Oncol 2007;5:106)

Treatment / prognosis
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● Nephrectomy
● Does not appear to recur (unless inadequate excision) or metastasize (Am J Surg Pathol 2003;27:658)

Gross description
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● 4-13 cm, unencapsulated, partially circumscribed, firm white tumor in renal pelvis / adjacent soft tissue with a gelatinous / myxoid appearance
● No hemorrhage, no necrosis

Gross images
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Polypoid lesion of renal pelvis and calyces

Micro description
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● Varies; patterns include myxoid vascular (similar to nodular fasciitis), compact spindle cell with lymphoplasmacytic aggregates and hypocellular keloid-like areas of fibrosis
● Spindle cells are bland and uniform
● Occasional foam cells, osseous metaplasia or mild nuclear atypia may be present
● No / rare mitoses

Micro images
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Spindle cells and inflammatory cells in myxoid stroma


Ill-defined spindle cell proliferation of renal pelvis


Spindle cells in loose fascicular pattern


Various images

   
Various stains: smooth muscle actin+, vimentin+, CD34 stains some spindle cells and vessels

Positive stains
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● Smooth muscle actin, HHF35, vimentin; variable CD34

Negative stains
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● ALK1, desmin, keratin, p53

Differential diagnosis
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● Fibromatosis: culture-like fibroblasts, infiltrative border, usually no inflammatory infiltrate
● Fibrous histiocytoma: storiform pattern of fibrohistiocytic cells, possibly giant cells
● Spindle cell sarcoma: spindle cells exhibit atypia

End of Kidney tumor - cysts, children, adult benign > Benign (usually) adult tumors > Inflammatory myofibroblastic tumor


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