Pan Cuixuan, Jiang Danxian, Huang Jing, Xu Zumin, Yang Donghong, Xue Fei, Huang Zhouliang, Xiao Lin, Zhou Suzhu, Yu Zhonghua
Department of Oncology, Kaiping Central Hospital, Jiangmen, China.
Department of Head and Neck Oncology, Affiliated Hospital of Guangdong Medical University, Zhanjiang, China.
AME Case Rep. 2024 Jun 5;8:71. doi: 10.21037/acr-23-147. eCollection 2024.
Primary malignancies of the cervical lymph nodes with special pathological characteristics are relatively uncommon in clinical settings, and there have been few reports on these tumors. The precise basis for their pathogenesis is poorly understood, and their diagnosis can be challenging. In addition, no clinically validated treatments have been established to date for affected patients.
Here, we describe a case of a 65-year-old male patient who exhibited the enlargement of several lateral and supraclavicular lymph nodes on the right side of his neck that presented as a large mass associated with a high fever and benign leukocytosis. He did not exhibit any relevant prior history. Radiological assessment revealed that this lesion was the primary tumor and that it has since spread to the liver. Histological assessment was unable to definitively classify the pathological characteristics of this tumor. Without any relevant morphological findings, immunohistochemical outcomes were not sufficiently specific to clarify the origin of these cells. When distinguishing it from similar sarcomas of the lymphohematopoietic system, it was found to not be typical of a histiocytic or dendritic cell tumor. Treatment to this patient was performed following multidisciplinary consultation and consisted of one course of a cyclophosphamide plus doxorubicin, vincristine, and dexamethasone regimen and two courses of the cyclophosphamide plus pirarubicin, vincristine, and dexamethasone regimen. However, the tumor exhibited minimal response to such treatment. While radiotherapy was proposed, the patient lacked confidence in the approach and declined treatment. He eventually developed severe tumor-associated complications. In the discussion section of this report, we detail and analyze the pathogenesis, diagnosis, and referential treatments of this rare malignancy.
This is the first report describing such a malignancy, and we hope that the publication of these findings can lead to the recognition of this tumor while supporting efforts to acquire greater experience in the diagnosis and treatment of affected patients.
具有特殊病理特征的颈部淋巴结原发性恶性肿瘤在临床中相对少见，关于这些肿瘤的报道也较少。其发病机制的确切基础尚不清楚，诊断具有挑战性。此外，迄今为止尚未为受影响的患者建立经过临床验证的治疗方法。
在此，我们描述了一名65岁男性患者的病例，该患者右侧颈部多个外侧和锁骨上淋巴结肿大，表现为伴有高热和良性白细胞增多的巨大肿块。他没有任何相关既往史。影像学评估显示该病变为原发性肿瘤，且已扩散至肝脏。组织学评估无法明确分类该肿瘤的病理特征。在没有任何相关形态学发现的情况下，免疫组化结果也不够特异，无法阐明这些细胞的起源。在将其与淋巴造血系统的类似肉瘤区分时，发现它不是典型的组织细胞或树突状细胞瘤。该患者在多学科会诊后接受了治疗，包括一个疗程的环磷酰胺加阿霉素、长春新碱和地塞米松方案以及两个疗程的环磷酰胺加吡柔比星、长春新碱和地塞米松方案。然而，肿瘤对这种治疗反应极小。虽然提出了放疗建议，但患者对该方法缺乏信心并拒绝治疗。他最终出现了严重的肿瘤相关并发症。在本报告的讨论部分，我们详细分析了这种罕见恶性肿瘤的发病机制、诊断和参考治疗方法。
这是描述此类恶性肿瘤的首例报告，我们希望这些发现的发表能够促使人们认识到这种肿瘤，同时支持在受影响患者的诊断和治疗方面积累更多经验的努力。
