Soft Tissue Tumors - Juvenile xanthogranuloma variant of fibrous histiocytoma

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Soft Tissue Tumors

Fibrohistiocytic tumors

Juvenile xanthogranuloma variant of fibrous histiocytoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 27 October 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Benign proliferative disorder of dendrocytes in skin, usually self-limited (Am J Surg Pathol 2003;27:579)

Terminology
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● Also called nevoxanthoendothelioma (eMedicine)
● See also topics in these chapters: Breast-nonmalignant, Eye-uvea and Liver-tumor

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 features
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● Skin of face or trunk, less commonly in subcutis, skeletal muscle, eye, peripheral nerve or testis
● Also liver, spleen, kidney; rarely intracranial (Am J Neuroradiol 2011;32:E132)
● 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 papules (Pediatr Dermatol 2009;26:238)
● 20 year old woman with “extranumerary nipple” of breast (Case of Week #5)
● 49 year old woman with lingual lesion (J Med Case Rep 2011 Jan 24;5:30)

Treatment and prognosis
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● Conservative excision (Am J Surg Pathol 2005;29:21)
● 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)
● Can be fatal if massive hepatic or CNS involvement

Gross description
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● Up to 2 cm, yellow-red or brown, papulonodular

Clinical images
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Smooth domed papule

Nodule over dorsum of tongue

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
● 0-2 mitotic figures per 10 HPF, rarely numerous
● Late - epidermis thins out, rete ridges become elongated
● Deep lesions - more cellular and monotonous with fewer Touton cells, can have more mitosis

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

48 year old woman

Touton type giant cells: ring (wreath) of nuclei surround foamy cytoplasm, with cytoplasm visible around nuclei

Langhans type giant cell (for comparison): nuclei form a horseshoe arrangement; may not be distinct from Touton type

Foreign body type giant cell (for comparison): nuclei have haphazard arrangement

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

Cytology description
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● Vague, granulomatous aggregates with monotonous CD68+ histiocytic cells (Acta Cytol 2007;51:473)

Positive stains
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● Vimentin CD68, HAM56, Factor XIIIa; also NKI-C3/CD63 (60%), lysozyme, alpha-1–antichymotrypsin

Negative stains
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● S100, CD1a, EMA, keratin, HMB45

Electron microscopy description
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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
● Spindle cell xanthogranuloma: storiform array of cells and Touton type giant cells

End of Soft Tissue Tumors > Fibrohistiocytic tumors > Juvenile xanthogranuloma variant of fibrous histiocytoma

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