title: Juvenile xanthogranuloma presenting as a large neck mass and ocular complications: a diagnostic and therapeutic dilemma.
abstract: Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis that originates from dendritic cells. Dendritic cells can accumulate in the skin of the head, neck, trunk, arms and legs. They may also involve other tissues such as the bones, lung, liver, heart, bone marrow, central nervous system, spleen and large intestine in rare cases. We report a rare case of juvenile xanthogranuloma in a 16-year-old girl who presented with a neck mass and left-sided ptosis 2.5 months previously. Excisional biopsy of the neck lesion revealed proliferated histiocytes admixed with numerous eosinophils and multinucleated giant cells that simulate eosinophilic granuloma; however, the histiocytes were negative for CD1a, CD123 and S-100 protein and positive for CD68 and CD14. The course of the disease led to treatment of the patient with chemotherapy, followed by low-dose radiotherapy.
authors.affiliation: Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Islamic Republic of Iran.
authors.forename: Shokouh Taghipour
authors.initials: ST
authors.lastname: Zahir
authors.forename: Naser Sefidrokh
authors.initials: NS
authors.lastname: Sharahjin
authors.forename: Hasanali
authors.initials: H
authors.lastname: Vahedian
authors.forename: Ali
authors.initials: A
authors.lastname: Akhavan
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001763:Blepharoptosis / Q000209:etiology*; D003937:Diagnosis, Differential; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D009333:Neck / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed; D014972:Xanthogranuloma, Juvenile / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*
chemical_list: D000970:Antineoplastic Agents; D005938:Glucocorticoids; D011241:Prednisone