A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease
Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently r...
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MDPI AG
2023-07-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/59/8/1381 |
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| author | Li-Ching Liu Yann-Guang Chen Nien-Tzu Liu Yi-Hao Chen Ke-Hung Chien |
| author_facet | Li-Ching Liu Yann-Guang Chen Nien-Tzu Liu Yi-Hao Chen Ke-Hung Chien |
| author_sort | Li-Ching Liu |
| collection | DOAJ |
| description | Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies. |
| first_indexed | 2024-03-10T23:45:44Z |
| format | Article |
| id | doaj.art-852c1449f239464f8433796d2a93a0a2 |
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| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-10T23:45:44Z |
| publishDate | 2023-07-01 |
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| spelling | doaj.art-852c1449f239464f8433796d2a93a0a22023-11-19T02:05:19ZengMDPI AGMedicina1010-660X1648-91442023-07-01598138110.3390/medicina59081381A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related DiseaseLi-Ching Liu0Yann-Guang Chen1Nien-Tzu Liu2Yi-Hao Chen3Ke-Hung Chien4Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, TaiwanDepartment of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, TaiwanDepartment of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, TaiwanDepartment of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, TaiwanDepartment of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, TaiwanMulticentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies.https://www.mdpi.com/1648-9144/59/8/1381multicentric Castleman diseaseIgG4-related diseaseorbital tumorhistopathology |
| spellingShingle | Li-Ching Liu Yann-Guang Chen Nien-Tzu Liu Yi-Hao Chen Ke-Hung Chien A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease Medicina multicentric Castleman disease IgG4-related disease orbital tumor histopathology |
| title | A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease |
| title_full | A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease |
| title_fullStr | A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease |
| title_full_unstemmed | A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease |
| title_short | A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease |
| title_sort | rare case of orbital castleman disease with overlapping igg4 related disease |
| topic | multicentric Castleman disease IgG4-related disease orbital tumor histopathology |
| url | https://www.mdpi.com/1648-9144/59/8/1381 |
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