Primary orbital yolk sac tumor in an infant: A rare entity

We report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonanc...

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Main Authors: Varsha Mishra, Sandeep Jain, Payal Malhotra, Garima Durga, Gauri Kapoor
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-07-01
Series:Indian Journal of Pathology and Microbiology
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Online Access:https://journals.lww.com/10.4103/IJPM.IJPM_741_21
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author Varsha Mishra
Sandeep Jain
Payal Malhotra
Garima Durga
Gauri Kapoor
author_facet Varsha Mishra
Sandeep Jain
Payal Malhotra
Garima Durga
Gauri Kapoor
author_sort Varsha Mishra
collection DOAJ
description We report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonance imaging (MRI) orbit revealed a lobulated heterogeneously enhancing right retroocular mass extending up to the orbital apex, displacing the optic nerve and eroding the medial orbital wall. Biopsy of the lesion revealed pure YST histology. Serum alpha-fetoprotein (AFP) was markedly raised at 76900 ng/mL. She was started on infant bleomycin etoposide cisplatin (BEP) chemotherapy protocol. There was a good clinical and radiological response. A high index of malignancy is required in young children presenting with orbital proptosis. A multidisciplinary approach and early intervention are essential to save both vision and life.
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institution Kabale University
issn 0377-4929
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publishDate 2023-07-01
publisher Wolters Kluwer Medknow Publications
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series Indian Journal of Pathology and Microbiology
spelling doaj-art-1a0e0daf0e72462b884059ea5dfba5542025-02-07T13:37:58ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292023-07-0166365265410.4103/IJPM.IJPM_741_21Primary orbital yolk sac tumor in an infant: A rare entityVarsha MishraSandeep JainPayal MalhotraGarima DurgaGauri KapoorWe report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonance imaging (MRI) orbit revealed a lobulated heterogeneously enhancing right retroocular mass extending up to the orbital apex, displacing the optic nerve and eroding the medial orbital wall. Biopsy of the lesion revealed pure YST histology. Serum alpha-fetoprotein (AFP) was markedly raised at 76900 ng/mL. She was started on infant bleomycin etoposide cisplatin (BEP) chemotherapy protocol. There was a good clinical and radiological response. A high index of malignancy is required in young children presenting with orbital proptosis. A multidisciplinary approach and early intervention are essential to save both vision and life.https://journals.lww.com/10.4103/IJPM.IJPM_741_21germ cell tumorinfantmalignancyorbit
spellingShingle Varsha Mishra
Sandeep Jain
Payal Malhotra
Garima Durga
Gauri Kapoor
Primary orbital yolk sac tumor in an infant: A rare entity
Indian Journal of Pathology and Microbiology
germ cell tumor
infant
malignancy
orbit
title Primary orbital yolk sac tumor in an infant: A rare entity
title_full Primary orbital yolk sac tumor in an infant: A rare entity
title_fullStr Primary orbital yolk sac tumor in an infant: A rare entity
title_full_unstemmed Primary orbital yolk sac tumor in an infant: A rare entity
title_short Primary orbital yolk sac tumor in an infant: A rare entity
title_sort primary orbital yolk sac tumor in an infant a rare entity
topic germ cell tumor
infant
malignancy
orbit
url https://journals.lww.com/10.4103/IJPM.IJPM_741_21
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AT payalmalhotra primaryorbitalyolksactumorinaninfantarareentity
AT garimadurga primaryorbitalyolksactumorinaninfantarareentity
AT gaurikapoor primaryorbitalyolksactumorinaninfantarareentity