Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Eur J Ophthalmol. 2022 Jan;32(1):NP246-NP250. doi: 10.1177/1120672120963459. Epub 2020 Nov 12.
Ocular adnexal lymphomas (OAL) constitute 55% of all orbital tumors. Waldenström Macroglobulinemia (WM) presenting as an orbital mass with diffuse extraocular muscle (EOM) involvement is rare. We report an elderly patient who presented to the ophthalmologist for an orbital mass which on evaluation, turned out to an ocular adnexal WM.
A 75 years old man presented with a palpable mass in the left anterior superior orbit and bilateral restricted ocular motility in all gazes. Computed tomography scan revealed a hyperdense mass with diffuse thickening of extraocular muscles and enlarged lacrimal gland on the left side. Incisional biopsy of the mass revealed a lymphoproliferative neoplasm with plasmacytic morphology. Immunohistochemistry (IHC) of the orbital mass as well as the bone marrow was sought, lymphoplasmacytic lymphoma (CD20+, CD38+, MUM1+, BCL 2+, CD3-, CD5-, CD10-, CD23-, cyclin D1). Bone marrow flow cytometry showed CD5-, CD10- kappa restricted B cell neoplasm. Serum analysis significantly elevated IgM levels. This indicated a diagnosis of ocular adnexal Waldenström Macroglobulinemia.
This case highlights the importance of clinical evaluation, histopathology, and immunohistochemistry for phenotyping of ocular adnexal lymphomas.
眼附属器淋巴瘤（OAL）占所有眼眶肿瘤的 55%。以弥漫性眼外肌（EOM）受累为表现的瓦尔登斯特伦巨球蛋白血症（WM）引起的眼眶肿块较为罕见。我们报告了 1 例老年患者，因眼眶肿块就诊于眼科，经评估，结果为眼附属 WM。
1 名 75 岁男性因左眼额上眶内可触及肿块和双侧眼球运动受限就诊。计算机断层扫描显示左侧高密度肿块，眼外肌弥漫性增厚，泪腺增大。肿块的切开活检显示为浆细胞形态的淋巴增生性肿瘤。对眼眶肿块和骨髓进行免疫组织化学（IHC）检查，结果为淋巴浆细胞淋巴瘤（CD20+，CD38+，MUM1+，BCL 2+，CD3-，CD5-，CD10-，CD23-，cyclin D1）。骨髓流式细胞术显示 CD5-，CD10-κ受限 B 细胞肿瘤。血清分析显著升高 IgM 水平。这表明诊断为眼附属器瓦尔登斯特伦巨球蛋白血症。
本病例强调了临床评估、组织病理学和免疫组织化学对眼附属器淋巴瘤表型分析的重要性。
