Atypical intravitreal growth of retinoblastoma with a multi-branching configuration
Purpose: To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration. Observations: A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching...
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Elsevier
2017-09-01
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Series: | American Journal of Ophthalmology Case Reports |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S2451993616301372 |
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author | Sachiko Nishina Satoshi Katagiri Atsuko Nakazawa Chikako Kiyotani Tadashi Yokoi Noriyuki Azuma |
author_facet | Sachiko Nishina Satoshi Katagiri Atsuko Nakazawa Chikako Kiyotani Tadashi Yokoi Noriyuki Azuma |
author_sort | Sachiko Nishina |
collection | DOAJ |
description | Purpose: To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration.
Observations: A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped calcification, and cystic lesions in that eye. Because it was difficult to rule out congenital anomalies such as persistent fetal vasculature due to the atypical clinical features of retinoblastoma, we performed a biopsy using a limbal approach. An intraoperative rapid pathological examination led to the definitive diagnosis of retinoblastoma. The right eye was enucleated and postoperative adjuvant chemotherapy was administered. Immunohistochemical staining of the enucleated eyeball showed that the tumoral cells and diaphanous tumoral tissue around the vessels were positive for neuron-specific enolase and Ki-67 and partially positive for glial fibrillary acidic protein (GFAP). The vessels of the diaphanous tissues near the tumoral mass were stained by GFAP and those behind the lens were stained faintly.
Conclusions and importance: We described an atypical retinoblastoma of pseudo-persistent fetal vasculature with a multi-branching configuration, which expanded the clinical spectrum of retinoblastoma. Such a specific growth pattern of the embryonic tumor might occur with a combination of retinal development, retinal vasculature, and hyaloid vascular system. |
first_indexed | 2024-12-20T04:12:16Z |
format | Article |
id | doaj.art-9803fb9646744a4ca3a24c3def46d59d |
institution | Directory Open Access Journal |
issn | 2451-9936 |
language | English |
last_indexed | 2024-12-20T04:12:16Z |
publishDate | 2017-09-01 |
publisher | Elsevier |
record_format | Article |
series | American Journal of Ophthalmology Case Reports |
spelling | doaj.art-9803fb9646744a4ca3a24c3def46d59d2022-12-21T19:53:52ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362017-09-017C4810.1016/j.ajoc.2017.04.003Atypical intravitreal growth of retinoblastoma with a multi-branching configurationSachiko Nishina0Satoshi Katagiri1Atsuko Nakazawa2Chikako Kiyotani3Tadashi Yokoi4Noriyuki Azuma5Department of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, Tokyo, JapanDepartment of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, Tokyo, JapanDepartment of Pathology, National Center for Child Health and Development, Tokyo, JapanChildren's Cancer Center, National Center for Child Health and Development, Tokyo, JapanDepartment of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, Tokyo, JapanDepartment of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, Tokyo, JapanPurpose: To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration. Observations: A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped calcification, and cystic lesions in that eye. Because it was difficult to rule out congenital anomalies such as persistent fetal vasculature due to the atypical clinical features of retinoblastoma, we performed a biopsy using a limbal approach. An intraoperative rapid pathological examination led to the definitive diagnosis of retinoblastoma. The right eye was enucleated and postoperative adjuvant chemotherapy was administered. Immunohistochemical staining of the enucleated eyeball showed that the tumoral cells and diaphanous tumoral tissue around the vessels were positive for neuron-specific enolase and Ki-67 and partially positive for glial fibrillary acidic protein (GFAP). The vessels of the diaphanous tissues near the tumoral mass were stained by GFAP and those behind the lens were stained faintly. Conclusions and importance: We described an atypical retinoblastoma of pseudo-persistent fetal vasculature with a multi-branching configuration, which expanded the clinical spectrum of retinoblastoma. Such a specific growth pattern of the embryonic tumor might occur with a combination of retinal development, retinal vasculature, and hyaloid vascular system.http://www.sciencedirect.com/science/article/pii/S2451993616301372RetinoblastomaAtypicalPersistent fetal vasculaturePathologyImmunohistochemistry |
spellingShingle | Sachiko Nishina Satoshi Katagiri Atsuko Nakazawa Chikako Kiyotani Tadashi Yokoi Noriyuki Azuma Atypical intravitreal growth of retinoblastoma with a multi-branching configuration American Journal of Ophthalmology Case Reports Retinoblastoma Atypical Persistent fetal vasculature Pathology Immunohistochemistry |
title | Atypical intravitreal growth of retinoblastoma with a multi-branching configuration |
title_full | Atypical intravitreal growth of retinoblastoma with a multi-branching configuration |
title_fullStr | Atypical intravitreal growth of retinoblastoma with a multi-branching configuration |
title_full_unstemmed | Atypical intravitreal growth of retinoblastoma with a multi-branching configuration |
title_short | Atypical intravitreal growth of retinoblastoma with a multi-branching configuration |
title_sort | atypical intravitreal growth of retinoblastoma with a multi branching configuration |
topic | Retinoblastoma Atypical Persistent fetal vasculature Pathology Immunohistochemistry |
url | http://www.sciencedirect.com/science/article/pii/S2451993616301372 |
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