464一例孤立性髓外鼻浆细胞瘤向眼眶局部扩展

P. Sandhar, C. Gill, A. Khanna
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摘要

摘要目的髓外浆细胞瘤是一种罕见的肿瘤,主要发生在头颈部,最常见的部位是鼻腔或鼻中隔。患者可能出现鼻出血、鼻塞或鼻漏。延伸到轨道是罕见的,但会导致视觉障碍。方法本病例报告涉及一位69岁女性患者,最初以6周的复发性鼻窦炎病史,左侧脸颊疼痛和复视。结果CT和MRI扫描显示左侧上颌窦、筛窦和蝶窦混浊,上颌内、前壁骨质破坏,并累及前软组织。肿块完全阻塞左鼻腔,并延伸至眶内侧。分期CT扫描也显示颈部结节性疾病。组织学活检CD138阳性,伴有不同成熟度和异型性的浆细胞弥漫性增殖。此外,单IgG kappa和CD45也呈阳性,提示淋巴样肿瘤。骨髓活检和PET扫描证实无全身累及。患者从COVID -19恢复后开始放射治疗。放射治疗6周后复查MRI扫描显示肿瘤缩小,眶内延伸消退,副蛋白消失,部分缓解。结论:本病例报告说明需要尽早考虑EMP诊断,以减少浆细胞瘤转化为多发性骨髓瘤的风险,这是一种已知的可能性,并确保最小延迟开始治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
464 Local Extension of a Solitary Extra Medullary Nasal Plasmacytoma into the Orbit
Abstract Aim Extramedullary plasmacytoma are rare neoplasms, which mainly occur in the head and neck, the most common site being the nasal cavity or septum. Patients may present with epistaxis, nasal obstruction, or rhinorrhoea. Extension into the orbit is rare but can lead to visual disturbances. Method This case report pertains to a 69-year-old female patient who initially presented with a 6-week history of recurrent sinusitis, with left sided cheek pain and diplopia. Results A CT and MRI scan was performed, which showed opacification of the left maxillary, ethmoid and sphenoid sinuses with bony destruction of the medial and anterior maxillary walls and into the anterior soft tissues. The mass was completely obstructing the left nasal cavity extending into the inferomedial orbit. Staging CT scans also showed nodal disease in the neck. Biopsy with histology was positive for CD138, with diffuse proliferation of plasma cells of varying maturity and atypia. Additionally, mono IgG kappa was also positive as was CD45 suggestive of a lymphoid neoplasm. Bone marrow biopsy and PET scan confirmed there was no systemic involvement. Radiotherapy was initiated after the patient recovered from COVID -19. A repeat MRI scan 6 weeks post radiotherapy showed a partial response with reduction in the size of the tumour, resolution of the intra-orbital extension and disappearance of paraprotein. Conclusions This case report illustrates need for earlier consideration of EMP diagnostically to reduce the risk of conversion of the plasmacytoma to multiple myeloma, which is a known possibility and to ensure minimal delay in the commencement of treatment.
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