Jumaat Anna Fariza, Mohamad Yunus Mohd Razif, Yong Doh Jeing, Md Zin Reena Rahayu, Mat Baki Marina
Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, University Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.
Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, 56000 Kuala Lumpur, Malaysia.
Iran J Otorhinolaryngol. 2023 Mar;35(127):101-108. doi: 10.22038/IJORL.2023.57806.2995.
An abnormal mass in the head and neck involving the supraglottic and cervical region offers a wide range of differential diagnoses. The pathology is either benign or malignant in nature. Castleman disease (CD) is an uncommon lymphoproliferative disorder characterised by hypervascular lymphoid hyperplasia and is classified into unicentric or multicentric disease. Histopathologically it is divided into hyaline vascular (HV), plasma cell (PC), and mixed cellularity variants. The multicentric disease is linked with PC and has the propensity to progress to lymphoma or Kaposi Sarcoma.
We report a case of a 45-year-old gentleman who presented with a painless anterior neck swelling and left supraglottic mass for six months. Computed tomography (CT) contrast imaging demonstrated a homogenous enhancing lesion at the left supraglottic and the midline of the anterior neck with erosive changes of the thyroid cartilage. A surgical resection of the anterior neck mass was performed. The diagnosis of Castleman disease plasma cell variant was made by histopathologic evaluation. The patient remained well post-resection.
Supraglottic multicentric Castleman disease is the least expected diagnosis in this case. Unicentric disease is treated with surgery. However, limited studies are available in determining the effectiveness of surgery in multicentric diseases. The plasma cell variant requires a multidisciplinary and multimodal approach due to an inclination towards malignancy. Research is needed to determine the role of surgery in multicentric disease and to develop optimum guidelines for managing cases. To date, there is unsubstantial literature describing supraglottic multicentric disease.
头颈部涉及声门上区和颈部区域的异常肿块有多种鉴别诊断。其病理性质可为良性或恶性。卡斯特尔曼病(CD)是一种罕见的淋巴增殖性疾病,其特征为血管增生性淋巴样增生,分为单中心型或多中心型疾病。组织病理学上可分为透明血管型(HV)、浆细胞型(PC)和混合细胞型。多中心型疾病与浆细胞型相关,有发展为淋巴瘤或卡波西肉瘤的倾向。
我们报告一例病例,一名45岁男性,出现无痛性颈部前方肿胀和左侧声门上区肿块6个月。计算机断层扫描(CT)增强成像显示左侧声门上区和颈部前方中线有一个均匀强化的病变,甲状软骨有侵蚀性改变。对颈部前方肿块进行了手术切除。通过组织病理学评估确诊为卡斯特尔曼病浆细胞型。患者术后恢复良好。
在本病例中,声门上区多中心型卡斯特尔曼病是最意想不到的诊断。单中心型疾病采用手术治疗。然而,关于确定手术在多中心型疾病中的有效性的研究有限。由于浆细胞型有恶变倾向,需要多学科和多模式的治疗方法。需要开展研究以确定手术在多中心型疾病中的作用,并制定最佳的病例管理指南。迄今为止,表示声门上区多中心型疾病的文献并不多。