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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
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● Benign, usually self-limited, proliferative disorder of dendrocytes in skin
Terminology
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● Also called nevoxanthoendothelioma
● See also Breast-nonmalignant, Eye-uvea and Liver-tumor chapters
Epidemiology
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● 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
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● 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
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● 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
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● 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
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● Up to 2 cm, yellow-red, papulonodular
Gross images
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Well circumscribed
yellow nodule
Other images: various images #1; #2
Dermoscopy description
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● Orange-yellow setting sun appearance (Dermatology 2007;215:256)
Micro description
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● 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
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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
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● Deep seated mass: vague, granulomatous aggregates with monotonous, CD68+ histiocytic cells (Acta Cytol 2007;51:473)
Positive stains
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● CD68, HAM56, Factor XIIIa; also NKI-C3/CD63 (60%)
Negative stains
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● S100, CD1a
Electron microscopy
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● No Birbeck granules, may have cytoplasmic lipid
Differential diagnosis
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● 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
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● AJSP 2003;27:579, AJSP 2005;29:21, eMedicine
End of Skin / Soft Tissue Tumors > Juvenile xanthogranuloma variant of fibrous histiocytoma
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