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Case Reports
. 2015 Dec 10;6(3):443-7.
doi: 10.1159/000442744. eCollection 2015 Sep-Dec.

Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule

Koji Kitazawa  1 , Kenji Nagata  2 , Yukito Yamanaka  2 , Yasumichi Kuwahara  3 , Tomoko Iehara  3 , Shigeru Kinoshita  4 , Chie Sotozono  2
Affiliations
Case Reports

Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule

Koji Kitazawa et al. Case Rep Ophthalmol. .

Abstract

Background: Retinoblastomas account for 4% of malignancies in children, 1-2% of which are diffuse infiltrating retinoblastomas. Diffuse anterior retinoblastoma is rare and does not involve the retina. Here, we report on a diffuse anterior retinoblastoma with large sarcoidosis-like nodules on the iris that were responsive to anti-inflammatory therapy.

Case: We present a 6-year-old girl who had anterior uveitis with white nodules on the iris and posterior surface of the cornea in her right eye. The nodules initially responded well to anti-inflammatory treatment. However, anterior segment optical coherence tomography (AS-OCT) showed that the nodules gradually grew, shrinking the iris. We then collected the aqueous humor for diagnosis. A biopsy revealed clusters of small cells with a high nuclear-to-cytoplasm ratio with partial rosette formation. Therefore, we diagnosed diffuse anterior retinoblastoma without retinal involvement and performed enucleation of the right eye. The histopathology demonstrated undifferentiated cells similar to those seen on the biopsy, and tumor cells invaded the iris stroma, posterior surface of the cornea, ciliary body, and sclera. After the enucleation, she underwent chemotherapy and remains alive.

Conclusion: A differential diagnosis of retinoblastoma should be considered when white nodules refractory to anti-inflammatory therapy occur in the eye, even in the absence of obvious retinal masses. AS-OCT findings are useful in assessing retinoblastoma.

Keywords: Anterior segment optical coherence tomography; Chemotherapy; Diffuse anterior retinoblastoma; Diffuse infiltrating retinoblastoma; Enucleation; Retinoblastoma; Sarcoidosis.

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Figures

Fig. 1
Fig. 1
a Initial slit-lamp examination of the right eye. White nodules are present on the iris and posterior surface of the cornea. b Slit-lamp examination performed prior to the biopsy. Nodules showed horizontal growth across the posterior surface of the cornea. c Image of the anterior segment on optical coherence tomography (SS-100 CASIA, TOMEY, Nagoya, Japan) performed prior to the biopsy.
Fig. 2
Fig. 2
a The biopsy showed clusters of small undifferentiated cells in a rosette formation. b Tumor cell infiltration in the iris (arrow).
See this image and copyright information in PMC

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