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Kidney tumor - adult malignancies

Other neoplasms – adult or adult/child

Angiosarcoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 26 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Malignant vascular tumor with anastomosing vascular spaces lined by atypical endothelial cells
● Very rare as primary kidney tumor (< 50 cases reported), almost all men, more often left-sided, presenting with hematuria, flank pain, or palpable mass; metastasis to the kidney from a primary site elsewhere (such as skin) is also rare
● Poor prognosis (Am J Surg Pathol 2010:34:942)
● Risk factors at other sites include exposure to arsenic, Thorotrast, polyvinyl chloride or radiation; also posttreatment lymphedema

Case reports
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● 38 year old woman with adult Wilms’ tumor and angiosarcoma (Cancer Chemother Pharmacol 2008;61:717)
● 50 year old man with kidney mass, liver and lung nodules (Arch Pathol Lab Med 2002;126:478)
● 61 year old man whose tumor had minute clear cell carcinomas (Pathol Res Pract 2009;205:347)
● Mixture of poorly differentiated spindled sarcoma and angiomatous differentiation (Arch Pathol Lab Med 1998;122:929)
● Occurrence in brothers at ages 52 and 69 years (Arch Pathol Lab Med 1995;119:75)

Gross images
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Multiple hemorrhagic masses within the renal parenchyma

Micro description
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● Infiltrative, often with extensive destruction of the parenchyma, at least small areas of vasoformative growth (Am J Surg Pathol 2010:34:942), although spindled/epithelioid growth may be prominent

Micro images
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Anastomosing vascular channels with minimal atypia in lining cells


Endothelial cells in compact sheets with abortive vascular spaces and moderate atypia


Numerous blood filled vascular spaces are lined by plump endothelial cells

Cytology description
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● Highly pleomorphic cells, some containing hemosiderin
● Neovascular structures with intracytoplasmic lumina containing erthrocytes
● Cells are non-cohesive with long cytoplasmic processes
● Erythrophagocytosis is present; variable cellularity due to necrosis

Cytology images
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50 year old man - various images

Positive stains
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● Vimentin, Factor VIII related antigen, CD34, CD31, FLI1, Ulex europaeus

Negative stains
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● Keratin, Cam 5.2, AE1/AE3, S100, CEA, EMA, desmin, smooth muscle actin, MelanA, HMB45

Electron macroscopy description
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● Spindle cells with abundant pinocytotic vesicles, intermediate filaments, rare Weibel-Palade bodies

Differential diagnosis
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Sarcomatoid renal cell carcinoma: usually a transition from an epithelial malignant component to high-grade sarcomatous component can be found on multiple sections; keratin+, CD31, CD34 negative; epithelioid angiosarcoma may be challenging, as keratin markers sometimes positive (Diagn Cytopathol 2011 Jun 22, Epub ahead of print, Am J Surg Pathol 2010:34:942)
Clear cell renal cell carcinoma: may also have prominent vascularity but usually classic areas of epithelioid, clear cell morphology can be seen, EMA+, keratin+
Angiomyolipoma (classic and epithelioid): positive for melanocytic markers such as MelanA and HMB45 (Am J Surg Pathol 2010:34:942)

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


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