Emperipolesis in a Juvenile Xanthogranuloma: Sentinel Case Report and Review of the Literature

Kurt J Knowles, Shoujun Chen, Kathryn Rhymes, Kevin Boykin and Ailing Li

Department of Pathology and Translational Pathobiology, LSU Health Science Center Shreveport, USA
Department of Pediatric Surgery, LSU Health Science Center enter Shreveport, USA

^*Correspondance to: Kurt J Knowles 

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Abstract:

This is the first reported case of a giant juvenile xanthogranuloma with multiple unusual features. The patient is an infant who presented with a rapidly growing 4.0 cm soft tissue mass on the back with necrosis and ulceration. MRI demonstrated the characteristic signaling of sebaceous cyst. Histology revealed a large and well-circumscribed dermal lesion that is composed predominately of histiocytes, lymphocytes, eosinophils, multinucleated giant cells, Touton giant cells and the extremely unique feature consisting of an extraordinarily high degree of emperipolesis. The diagnosis of juvenile xanthogranuloma is confirmed by positive immunostaining for CD68 and CD163, and negative immunostaining for CD1a, CD117 and S100 ruled out a Langerhans cell lesion. The final diagnosis is giant juvenile xanthogranuloma with emperipolesis simulating Rosai-Dorfman Disease, a lesion that has never been reported, and may represent a mixture of a dendritic cell related tumor and a macrophage related tumor according to the 1997 WHO classification of histiocytic lesions.

Keywords:

Xanthogranuloma; Juvenile; Emperipolesis; Non-Langerhans cell histiocytosis

Cite the Article:

Knowles KJ, Chen S, Rhymes K, Boykin K, Li A. Emperipolesis in a Juvenile Xanthogranuloma: Sentinel Case Report and Review of the Literature. Ann Clin Case Rep. 2018; 3: 1559.