A Rare Presentation of Orbital Castleman’s Disease

Castleman’s disease (CD) is an uncommon group of atypical lymphoproliferative disorders. Extranodal involvement such as the orbit is extremely rare. We aim to report a case of a 62-year-old male who presented with left painless proptosis for the past three years. Examination revealed a firm, lobulat...

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Main Authors: Ruchi Goel, Akash Raut, Ayushi Agarwal, Shweta Raghav, Sumit Kumar, Simmy Chaudhary, Priyanka Golhait, Sushil Kumar, Ravindra Saran
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2020/1012759
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author Ruchi Goel
Akash Raut
Ayushi Agarwal
Shweta Raghav
Sumit Kumar
Simmy Chaudhary
Priyanka Golhait
Sushil Kumar
Ravindra Saran
author_facet Ruchi Goel
Akash Raut
Ayushi Agarwal
Shweta Raghav
Sumit Kumar
Simmy Chaudhary
Priyanka Golhait
Sushil Kumar
Ravindra Saran
author_sort Ruchi Goel
collection DOAJ
description Castleman’s disease (CD) is an uncommon group of atypical lymphoproliferative disorders. Extranodal involvement such as the orbit is extremely rare. We aim to report a case of a 62-year-old male who presented with left painless proptosis for the past three years. Examination revealed a firm, lobulated mass in the left superotemporal orbit, displacing the globe inferomedially. A well-defined extraconal orbital lesion encasing the left lateral rectus muscle with intraconal extension was seen on Magnetic Resonance Imaging (MRI) that led to the provisional diagnosis of left solitary encapsulated venous malformation. Excision of the mass via lateral orbitotomy was performed. However, on histopathology, the features were consistent with a mixed-cell variant of Castleman’s disease. A detailed systemic workup was unremarkable. Proptosis resolved after surgery and no recurrence was noted in the three-year follow-up. To the best of our knowledge, this is the first case report of a mixed-cell variant of unicentric orbital CD without any systemic features. This case highlights the importance of including CD in the differential diagnosis of well-defined orbital lesions so as to enable its early detection and timely management.
format Article
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institution Kabale University
issn 2090-6722
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language English
publishDate 2020-01-01
publisher Wiley
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series Case Reports in Ophthalmological Medicine
spelling doaj-art-918a666c753e447ab4026380172b20172025-02-03T06:46:00ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302020-01-01202010.1155/2020/10127591012759A Rare Presentation of Orbital Castleman’s DiseaseRuchi Goel0Akash Raut1Ayushi Agarwal2Shweta Raghav3Sumit Kumar4Simmy Chaudhary5Priyanka Golhait6Sushil Kumar7Ravindra Saran8Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaKallam Anji Reddy Campus, L V Prasad Marg, Banjara Hills, Hyderabad 500034, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, IndiaDepartment of Pathology, Govind Ballabh Pant Post Graduate Institute of Education and Medical Research, New Delhi 110002, IndiaCastleman’s disease (CD) is an uncommon group of atypical lymphoproliferative disorders. Extranodal involvement such as the orbit is extremely rare. We aim to report a case of a 62-year-old male who presented with left painless proptosis for the past three years. Examination revealed a firm, lobulated mass in the left superotemporal orbit, displacing the globe inferomedially. A well-defined extraconal orbital lesion encasing the left lateral rectus muscle with intraconal extension was seen on Magnetic Resonance Imaging (MRI) that led to the provisional diagnosis of left solitary encapsulated venous malformation. Excision of the mass via lateral orbitotomy was performed. However, on histopathology, the features were consistent with a mixed-cell variant of Castleman’s disease. A detailed systemic workup was unremarkable. Proptosis resolved after surgery and no recurrence was noted in the three-year follow-up. To the best of our knowledge, this is the first case report of a mixed-cell variant of unicentric orbital CD without any systemic features. This case highlights the importance of including CD in the differential diagnosis of well-defined orbital lesions so as to enable its early detection and timely management.http://dx.doi.org/10.1155/2020/1012759
spellingShingle Ruchi Goel
Akash Raut
Ayushi Agarwal
Shweta Raghav
Sumit Kumar
Simmy Chaudhary
Priyanka Golhait
Sushil Kumar
Ravindra Saran
A Rare Presentation of Orbital Castleman’s Disease
Case Reports in Ophthalmological Medicine
title A Rare Presentation of Orbital Castleman’s Disease
title_full A Rare Presentation of Orbital Castleman’s Disease
title_fullStr A Rare Presentation of Orbital Castleman’s Disease
title_full_unstemmed A Rare Presentation of Orbital Castleman’s Disease
title_short A Rare Presentation of Orbital Castleman’s Disease
title_sort rare presentation of orbital castleman s disease
url http://dx.doi.org/10.1155/2020/1012759
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