Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Kidney tumor - cysts, children, adult benign

Childhood neoplasms

Anaplastic sarcoma


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 30 March 2012, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
=========================================================================

● Rare tumor, first described in 2007 (Am J Surg Pathol 2007;31:1459)
● Characterized by a proliferation of anaplastic spindle cells with bizarre, pleomorphic nuclei and atypical mitotic figures
● May show chondroid differentiation

Epidemiology
=========================================================================

● Age range 1–41 years (median: 5 years; mean: 12 years) with slight female predominance

Case reports
=========================================================================

● 12 year old girl with chromosomal abnormalities in tumor (Hum Pathol 2010;41:1495)
● 24 year old woman (ScientificWorldJournal 2009;9:97)

Clinical features
=========================================================================

● Presents with a renal mass at an advanced stage
● Metastases to liver, lung and bone

Gross description
=========================================================================

● Large (mean: 12 cm) tumors, usually with cystic component
● Almost half involve pelvi-calyceal system

Micro description
=========================================================================

● Fascicular pattern of spindle cells with anaplastic changes diffusely or in multiple foci (bizarre pleomorphic cells and atypical mitotic figures)
● Foci of chondroid differentiation with both benign and malignant features, including islands of hyaline cartilage or chondroid matrix
● Occasional foci of osteoid and primitive blastema-like areas
● No epithelial structures, no nephrogenic rests

Micro images
=========================================================================


Spindle cells in collagenous stroma, also anaplastic cells in cellular focus

Positive stains
=========================================================================

● Vimentin, desmin and often p53

Negative stains
=========================================================================

● CAM 5.2, MyoD1, CD34, CD99 and WT1

Molecular description
=========================================================================

● Absence of synovial sarcoma t(X;18) and mesoblastic nephroma’s t(12;15) ETV6-NTRK3 translocation

Differential diagnosis
=========================================================================

Anaplastic Wilms’ tumor: prominent blastema component, foci of epithelial differentiation, occasionally nephrogenic rests and no very atypical and wildly anaplastic cells in stroma and cartilage; blastema areas are WT1+ and CD56+
Sarcomatoid carcinoma: has keratin+ epithelial component
Primary renal synovial sarcoma: lacks t(X;18)


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).