Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report.

Acta Neuropathologica Communications
Nishanth S IyengarChristopher William

Abstract

Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distincti...Continue Reading

References

Mar 1, 1979·American Journal of Ophthalmology·E FoucarR F Dorfman
Feb 23, 1999·Radiologic Clinics of North America·L T Bilaniuk
Apr 28, 2006·Blood·Didier DecaudinLivia Lumbroso-Le Rouic
May 23, 2006·Ophthalmic Plastic and Reconstructive Surgery·Yasaman MohadjerPhilip L Custer
May 24, 2007·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Yoh ZenYasuni Nakanuma
Jul 1, 2008·Journal of Hematology & Oncology·Geeta K VemugantiSantosh G Honavar
Jun 16, 2010·Nature Reviews. Gastroenterology & Hepatology·Terumi KamisawaTsuneo Sasaki
Nov 16, 2011·Head & Neck·Kwong Lun LaiC Andrew van Hasselt
May 19, 2012·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Vikram DeshpandeJohn H Stone
Apr 19, 2013·American Journal of Clinical Pathology·Xuefeng ZhangJames Vardiman
Aug 21, 2013·American Journal of Clinical Pathology·Liping LiuKai Fu
Jun 3, 2014·Indian Journal of Ophthalmology·Vibha DuttaPoninder Kumar
Feb 11, 2015·Annals of the Rheumatic Diseases·Mollie N CarruthersJohn H Stone
Mar 26, 2015·Head and Neck Pathology·Vikram Deshpande
Mar 27, 2015·Arthritis & Rheumatology·A KhosroshahiUNKNOWN Second International Symposium on IgG4-Related Disease
Jun 21, 2015·Journal of Otolaryngology - Head & Neck Surgery = Le Journal D'oto-rhino-laryngologie Et De Chirurgie Cervico-faciale·Graeme B MulhollandDavid W J Côté
Oct 13, 2015·Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons·Taiseer Hussain Hassan Al-Khateeb
Mar 26, 2016·Pediatric Rheumatology Online Journal·Faiz KarimJan van Laar
May 8, 2016·Radiologic Clinics of North America·Venkateswar R SurabhiSrinivasa R Prasad
Dec 22, 2017·Brain Pathology·Luca BerteroPaola Cassoni
May 15, 2018·Saudi Journal of Ophthalmology : Official Journal of the Saudi Ophthalmological Society·Diego StrianeseGiuseppe Mariniello
Aug 21, 2018·Ophthalmic Plastic and Reconstructive Surgery·Andrea A TooleyJames A Garrity
Dec 19, 2018·The Journal of Laryngology and Otology·G TirelliG C Del Piero
Feb 9, 2019·Acta Ophthalmologica·Sanne E DetigerDion Paridaens
Jul 6, 2019·Survey of Ophthalmology·Ilse MombaertsSteffen Heegaard

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Methods Mentioned

BETA
biopsy
biopsies
dissection

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