Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, Taiwan.
Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei 11490, Taiwan.
Medicina (Kaunas). 2023 Jul 28;59(8):1381. doi: 10.3390/medicina59081381.
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.
多中心Castleman 病（MCD）是一种全身性淋巴组织增生性疾病，可导致肺部、肾脏和结外部位等多种身体部位出现肿块。同时，眼眶 Castleman 病极为罕见。免疫球蛋白 G4 相关疾病（IgG4-RD）是一种新认识的纤维炎性疾病，其特征是形成具有淋巴浆细胞浸润的肿瘤样病变，富含 IgG4 阳性浆细胞，可能存在不同程度的特征性席纹状纤维化模式。在本研究中，我们报告了一例 67 岁台湾男性，双侧眼睑肿胀和眼球突出 7 年。眼眶磁共振成像显示双侧眶内区软组织病变，泪腺明显强化，并向双侧眶下孔延伸，提示眼眶淋巴组织增生性疾病。眼眶内肿瘤切除的组织病理学结果提示为浆细胞为主的混合细胞型 MCD。然而，该患者还表现出明确的 IgG4-RD 征象，包括泪腺肿大和浆细胞增多、纤维化和生发中心的组织病理学结果，IgG4 细胞比例增加和血清 IgG4 水平升高。该病例提示这两种疾病实体之间存在潜在的相互作用途径，需要进一步研究。
