Kidney tumor - cysts, children, adult benign - Sinus histiocytosis with massive lymphadenopathy

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Kidney tumor - cysts, children, adult benign

Benign (usually) adult tumors

Sinus histiocytosis with massive lymphadenopathy

Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 29 March 2012, last major update March 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Histiocytic disorder also known as Rosai-Dorfman disease (see also Lymph nodes–not lymphoma chapter)

Epidemiology
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● Rare disease with even rarer involvement of kidney
● More common in children and young adults

Clinical features
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● Fever, leukocytosis, anemia, elevated sedimentation rate and polyclonal hypergammaglobulinemia
● Relatively favorable prognosis
● Poor prognostic factors include multi-organ involvement, disseminated nodal disease and systemic immunologic disorder

Case reports
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● 13 year old boy with large, asymptomatic renal mass with adjacent hilar adenopathy (Arch Pathol Lab Med 1985;109:867)
● 13 year old boy with extensive involvement of multiple organ systems, leading to death (Arch Pathol Lab Med 1982;106:13)
● 31 year old man with generalized AA amyloidosis (Hum Pathol 2000;31:621)
● 37 year old with large bilateral masses of renal hilum (Arch Pathol Lab Med 1992;116:1366)
● 39 year old man with hematuria (Ann Saudi Med 2009;29:55)
● 76 year old man with prostate carcinoma (Arch Pathol Lab Med 2006;130:1223)

Gross description
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● Pelvic / sinus mass with multi-nodular, tan-yellow or gray hemorrhagic cut surface (may appear similar to renal cell carcinoma)

Gross images
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Well circumscribed mass with a creamy-brownish appearance

Micro description
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● Tissue infiltration by sheets of large histiocytes with granular eosinophilic cytoplasm, large, round and vesicular nuclei, prominent nucleoli, and accompanied by plasma cells and lymphocytes
● Extensive emperipolesis (histiocytes with intracytoplasmic lymphocytes)

Micro images
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Emperipolesis in various disorders

Lymphoid infiltrate surrounding scattered large histiocytic cells containing phagocytosed intracytoplasmic lymphocytes (arrows)

Various images

S100+

Positive stains
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● S100

Negative stains
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● CD1a

End of Kidney tumor - cysts, children, adult benign > Benign (usually) adult tumors > Sinus histiocytosis with massive lymphadenopathy

Ref Updated: 3/12/12

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