Permaisuari Nizma, Anggraini Neni, Mahyuddin Mutmainah, Kodrat Evelina, Rajabto Wulyo
Department of Ophthalmology, Faculty of Medicine Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Department of Anatomic Phatology, Faculty of Medicine Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Ocul Oncol Pathol. 2023 Feb;8(4-6):197-202. doi: 10.1159/000527273. Epub 2022 Oct 12.
Orbital plasmacytoma is a tumor of plasma cells located in the orbit, which is uncommon and only accounts for less than 1% of total orbital tumors. Sixty-five percent of orbital plasmacytoma are carrying a diagnosis of multiple myeloma. We hereby present two aggressive orbital plasmacytoma cases, a rare orbital malignancy with unsatisfactory outcomes.
This is a series of two orbital plasmacytoma cases. Both initial complaints were unilateral rapid onset of non-axial proptosis with palpable mass in the superior orbit. The first case was IgA-type multiple myeloma with multiple secondary plasmacytomas diagnosed based on systemic evaluation showing hyperproteinemia, IgA level elevation with free κ-light chains, and multiple destructive osteolytic lesions. The second patient unfortunately died before systemic evaluation was carried out.
Both patients died less than 2 months after diagnosis, underscoring a very poor prognosis. It is important to perform systemic evaluation and appropriate treatment immediately once the diagnosis has been established.
Orbital plasmacytoma is a rare orbital malignancy and is commonly secondary to systemic multiple myeloma. Ophthalmologists should have a high index of suspicion as it has a nonspecific presentation and consider it as one of the differential diagnoses in orbital tumors.
眼眶浆细胞瘤是一种位于眼眶的浆细胞肿瘤，较为罕见，仅占眼眶肿瘤总数的不到1%。65%的眼眶浆细胞瘤伴有多发性骨髓瘤的诊断。我们在此报告两例侵袭性眼眶浆细胞瘤病例，这是一种罕见的眼眶恶性肿瘤，预后不佳。
这是一系列两例眼眶浆细胞瘤病例。两例患者最初的主诉均为单侧眼眶迅速出现非轴性眼球突出，眼眶上部可触及肿块。第一例为IgA型多发性骨髓瘤伴多发继发性浆细胞瘤，通过全身评估诊断，表现为高蛋白血症、IgA水平升高伴游离κ轻链，以及多个破坏性溶骨性病变。第二例患者不幸在进行全身评估前死亡。
两名患者均在诊断后不到2个月内死亡，预后极差。一旦确诊，立即进行全身评估和适当治疗非常重要。
眼眶浆细胞瘤是一种罕见的眼眶恶性肿瘤，通常继发于系统性多发性骨髓瘤。眼科医生应高度怀疑，因其表现不具特异性，并将其作为眼眶肿瘤的鉴别诊断之一。
