Home Chapter Home Jobs Conferences Fellowships Books

Skin / Soft Tissue Tumors

Juvenile xanthogranuloma variant of fibrous histiocytoma

Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.

Reviewer: David Lucas, M.D., University of Michigan Health Systems (January 2009)

Revised: 22 October 2009, last major update June 2009

Definition

=========================================================================

● Benign, usually self-limited, proliferative disorder of dendrocytes in skin

Terminology

=========================================================================

● Also called nevoxanthoendothelioma

● See also Breast-nonmalignant, Eye-uvea and Liver-tumor chapters

Epidemiology

=========================================================================

● Uncommon (< 0.5% of pediatric tumors in one study)

● Usually infants (median age 5 months) with a congenital mark, although 10-30% occur in adults

● Male/female = 1.4:1

Clinical

=========================================================================

● Skin of face or trunk, less commonly in subcutis, skeletal muscle, eye, peripheral nerve or testis

● 20% of patients have multiple lesions, usually males

● In neonates, rarely associated with giant cell hepatitis and tumor in liver and viscera, requiring chemotherapy

Case reports

=========================================================================

● 2 year old boy with multiple lichenoid juvenile xanthogranuloma (Pediatr Dermatol 2009;26:238)

● 20 year old woman with “extranumerary nipple” of breast (Case of Week #5)

Treatment and prognosis

=========================================================================

● Conservative excision

● May spontaneously regress leaving depressed area of skin with variable hyperpigmentation

● Multisystemic disease requires Langerhans cell histiocytosis-type chemotherapy (Pediatr Blood Cancer 2008;51:130)

Gross description

=========================================================================

● Up to 2 cm, yellow-red, papulonodular

Gross images

=========================================================================

Well circumscribed

yellow nodule

Other images: various images #1;  #2

Dermoscopy description

=========================================================================

● Orange-yellow setting sun appearance (Dermatology 2007;215:256)

Micro description

=========================================================================

● Dense dermal infiltrate of lymphocytes, histiocytes, Touton giant cells (usually), eosinophils and neutrophils, which may extend into subcutis

● Late - epidermis thins out, rete ridges become elongated

● Deep lesions - more cellular and monotonous with fewer Touton cells

Micro images

=========================================================================

20 year old woman (Case of the Week #5)

Adult patient                                                        Touton giant cell

Comparison of histiocytic giant cell types:

Touton type: ring (wreath)                Langhans type: nuclei form                             Foreign body type:

of nuclei surround foamy                 a horseshoe arrangement; may                     nuclei have haphazard

cytoplasm, with cytoplasm              not be distinct from Touton type                    arrangement

also visible around nuclei                Touton type

Other images: foam cells, Touton giant cells and scattered lymphocytes #1;  #2

Cytology description

=========================================================================

● Deep seated mass: vague, granulomatous aggregates with monotonous, CD68+ histiocytic cells (Acta Cytol 2007;51:473)

Positive stains

=========================================================================

● CD68, HAM56, Factor XIIIa; also NKI-C3/CD63 (60%)

Negative stains

=========================================================================

● S100, CD1a

Electron microscopy

=========================================================================

● No Birbeck granules, may have cytoplasmic lipid

Differential diagnosis

=========================================================================

● Langerhans cell histiocytosis - more common, tumor cells have coffee bean nuclei/nuclear grooves, no Touton giant cells, are S100+ and CD1a+ and negative for CD68, HAM56 and Factor XIIIa, have Birbeck granules by EM

● Xanthoma - associated with hyperlipidemia, uniform collection of foam cells and variable Touton giant cells, but no other inflammatory cells

Additional references

=========================================================================

● AJSP 2003;27:579, AJSP 2005;29:21, eMedicine

End of Skin / Soft Tissue Tumors > Juvenile xanthogranuloma variant of fibrous histiocytoma

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must also 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, (c) 2001-2009, PathologyOutlines.com, Inc.  Information from third parties may also be protected by copyright.  Please contact us at copyrightPathOut@gmail.com with any questions.