Department of Pathology, The Third Affiliated Hospital, Sun Yat-Sen University, No. 600 Tianhe Road, Guangzhou, 510630, China.
J Cancer Res Clin Oncol. 2024 Jul 6;150(7):337. doi: 10.1007/s00432-024-05873-5.
Nasopharyngeal amyloidoma is a rare, locally aggressive tumor that has been reported in the English literature in only 38 cases to date, most of which were in the form of case reports. The present study was aimed to summarize the characteristics of this rare tumor, with the goal of providing new insights for diagnosis and treatment.
We report three cases of nasopharyngeal amyloidoma diagnosed in our hospital following comprehensive medical examination and review the current literature on all cases of nasopharyngeal amyloidoma from PubMed. The journey of nasopharyngeal amyloidoma, including presentation, diagnostics, surgeries, and follow-up was outlined.
None of the three patients had systemic amyloidosis. CT and nasal endoscopy showed irregular masses obstructing the nasopharyngeal cavity. Congo red staining confirmed the deposition of amyloid, and immunohistochemical analysis showed that the amyloid deposition was the AL light chain type. Through literature review, we found that nasopharyngeal amyloidoma most commonly occurred in individuals over the age of 40, patients usually had a good prognosis after complete tumor resection; however, there were still cases of recurrence, and unresected patients were at risk of progression to systemic amyloidosis. The efficacy of radiotherapy and chemotherapy was currently uncertain.
Early clinical and pathological diagnosis is crucial, and surgical intervention is the primary treatment option for this disease. Although patients usually have a favorable prognosis, long-term monitoring is necessary to detect potential relapses and initiate timely intervention.
鼻咽部淀粉样瘤是一种罕见的局部侵袭性肿瘤,迄今为止,仅在英文文献中报道了 38 例,且大多数为病例报告形式。本研究旨在总结这种罕见肿瘤的特征,为其诊断和治疗提供新的见解。
我们报告了我院诊断的 3 例鼻咽部淀粉样瘤病例,通过全面的医学检查,并复习了 PubMed 上所有鼻咽部淀粉样瘤的病例文献。概述了鼻咽部淀粉样瘤的发病过程,包括其表现、诊断、手术和随访。
这 3 例患者均无系统性淀粉样变性。CT 和鼻内镜检查显示不规则肿块阻塞鼻咽腔。刚果红染色证实了淀粉样物质的沉积,免疫组化分析显示淀粉样沉积为 AL 轻链型。通过文献复习,我们发现鼻咽部淀粉样瘤最常见于 40 岁以上人群,患者在完整切除肿瘤后通常预后良好;但仍有复发病例,未切除的患者有进展为系统性淀粉样变性的风险。目前,放化疗的疗效尚不确定。
早期的临床和病理诊断至关重要,手术干预是该疾病的主要治疗选择。尽管患者通常预后良好,但需要长期监测以发现潜在的复发并及时干预。