Successful treatment of juvenile xanthogranuloma using bevacizumab

PurposeTo report a case of juvenile xanthogranuloma involving the iris and skin that clincally was diagnosed with an obvious cutaneous lesion.ObservationsA four month-old girl with hyphema and increased intraocular pressure of the left eye persisting for 2 weeks. A suspicious yellow-brown mass with nodular surface and traversed by irregular vascularization was noted on the inferior iris surface. Ultrasound biomicroscopy (UBM; 35 MHz) of the mass revealed multiple nodular irregular hyperreflective lesions in the peripheral iris. Using a biopsy of an obvious cutaneous abdominal skin lesion a diagnosis was made based on histopathological analyses. The biopsy showed dense dermal infiltrate consisting of foamy histiocytes. Additional stains revealed CD68 positivity and CD1a and S100 negativity. This mass revealed histopathologic features identical to juvenile xanthogranuloma and was concurrent with the iris lesion. Next-generation sequencing using Ion AmpliSeqTM Cancer Hotspot Panel revealed a missense mutation of FGFR3 (p.F386L).Conclusion and importanceThe diagnosis of a xanthogranuloma of the iris with hyphema can be made easier in patients with obvious cutaneous lesions as described in our case. The significance of FGFR3 mutation in association with JXG is unknown and should be further investigated.

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“…The authors suggest that intraocular bevacizumab has a lower risk of cataract formation and secondary glaucoma than topical corticosteroids. 15 …”

Section: Discussionmentioning

confidence: 99%

Juvenile xanthogranuloma involving concurrent iris and skin: Clinical, pathological and molecular pathological evaluations

Meyer

Graeff

Kohler

et al. 2018

American Journal of Ophthalmology Case Reports

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“…Ashkenazy et al. (2014 13 ) treated two children with off-label intravitreal bevacizumab (1.25 mg/0.05 ml) with the aim to modulate the intrinsic vascularity of juvenile xanthogranuloma lesions followed by low-dose plaque radiotherapy of 35 Gy over four days, which led to a flat scar without recurrence of the disease.…”

Section: Discussionmentioning

confidence: 99%

Intraocular juvenile xanthogranuloma of the iris in an adult patient

Flockerzi

Häger

Seitz

2017

American Journal of Ophthalmology Case Reports

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PurposeJuvenile xanthogranuloma (JXG) is a rare histiocytic skin disease primarily of young children, which may also affect ocular structures and in particular the iris.ObservationsThis is a case report of a fifty-year-old patient without skin lesions showing a progressive decrease of visual acuity, iris vascularization and a yellowish iris tumor in the iridocorneal angle of his right eye. Treatment with topical and systemic prednisolone led to full recovery of visual acuity, tumor regression and restitutio ad integrum.Conclusions and importanceAlthough the juvenile xanthogranuloma is a very rare skin disease of young children, it may also affect the eye and in particular the iris in adult patients. There exists no standard treatment, the first-line therapy in most cases, however, is topical and systemic prednisolone application.

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“…При отсутствии эффекта в течение нескольких недель показано системное введение стероидных препаратов. При рефрактерности опухоли к любому виду введения ГКС N. Ashkenazy и соавторы предложили применять интраокулярные инъекции бевацизумаба (авастин) [21]. При мультифокальном или билатеральном поражении глаза, рефрактерном к другим видам лечения, применяют цитостатики (метотрексат, лейкеран, винбластин) [20,22,23].…”

Section: рис 5 юкг радужки при первичном обращении (а) и через 1 меunclassified

Juvenile xanthogranuloma of the eye

et al. 2018

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Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. JX can develop in any part of the eye and its appendages. Taking into consideration how rare this pathology is, its diagnosis and treatment are complicated. The article reviews 2 clinical cases of JX observed by the authors.

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Successful treatment of juvenile xanthogranuloma using bevacizumab

Cited by 25 publications

References 8 publications

Juvenile xanthogranuloma involving concurrent iris and skin: Clinical, pathological and molecular pathological evaluations

Juvenile xanthogranuloma involving concurrent iris and skin: Clinical, pathological and molecular pathological evaluations

Intraocular juvenile xanthogranuloma of the iris in an adult patient

Juvenile xanthogranuloma of the eye

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