Veisi Amirreza, Daneshvar Kimia, Hooshmandi Sadid, Najafi Maryam, Mohammadi Torbati Peyman, Hassanpour Kiana
Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Case Rep Ophthalmol. 2024 Mar 25;15(1):265-272. doi: 10.1159/000538120. eCollection 2024 Jan-Dec.
Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle.
A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation.
Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.
多发性骨髓瘤（MM）是一种浆细胞恶性肿瘤，是一种影响身体各个器官的全身性疾病。骨浆细胞瘤和髓外疾病（EMD）是MM的表现形式。EMD通常是该疾病更具侵袭性形式的标志。在此，我们报告一例难治性MM患者，其表现为上斜肌（SO）出现髓外浆细胞瘤。
一名51岁女性前来就诊，主诉20天前左眼逐渐突出并伴有眼痛。她1年前接受了骨髓移植，在过去1年中一直在接受MM化疗方案。眼部检查发现左眼突出，内收和上抬轻度受限。眼眶磁共振成像显示左SO肌明显增大，有局灶性对比增强。患者接受了活检和肿物减积手术。组织病理学检查显示纤维肌肉组织中含有一种肿瘤，该肿瘤由不同程度分化的成片浆细胞样细胞组成，其间有血管化程度较低的间质成分。浆细胞样细胞弥漫性表达分化簇138（CD138），从而诊断为累及眼外肌和眼眶软组织的EMD。患者接受了姑息性放疗和全身检查。PET-CT扫描显示骨盆骨和左小腿受累。因此，化疗方案升级以反映疾病的侵袭性。在最后一次随访中，眼眶软组织未见肿瘤复发迹象。不幸的是，患者在最近一次就诊7个月后因病去世。
早期识别疾病复发对MM患者的生命至关重要；眼部表现应被视为MM活动的一个迹象而予以认真考虑。
