V Vinay, Bansal Saraansh, Purwar Pallavi, Dewan Ravindra Kumar, Verma Paras, Mullick Shalini, Dixit Mallika, Phanindra B Rama, Siddhartha Kanishk
Department of Tuberculosis and Respiratory Diseases, National Institute of Tuberculosis and Respiratory Diseases (NITRD), New Delhi.
Department of Thoracic Surgery, National Institute of Tuberculosis and Respiratory Diseases (NITRD), New Delhi.
Monaldi Arch Chest Dis. 2022 Sep 20;93(2). doi: 10.4081/monaldi.2022.2360.
Follicular dendritic cell sarcoma is a rare low grade malignant neoplasm that arises from follicular dendritic cells in lymphoid tissue germinal centres and accounts for 0.4% of all soft tissue sarcomas. It is extremely rare to have pulmonary follicular dendritic cell sarcoma with endobronchial extension and as an anterior mediastinal mass with mediastinal lymph node involvement. We present the case of a 34-year-old male non-smoker who had been experiencing chest pain for three months. A lobulated left peri-hilar mass with endobronchial spread into the left main bronchus and mediastinal lymphadenopathy was identified on a chest CT. The bronchoscope-guided cryobiopsy of the endobronchial mass was inconclusive. After a thorough multidisciplinary discussion, the patient underwent left sided pneumonectomy, mediastinal mass resection, and systematic lymph node dissection. Histologic examination using immunohistochemistry revealed follicular dendritic cell sarcoma.
滤泡性树突状细胞肉瘤是一种罕见的低级别恶性肿瘤，起源于淋巴组织生发中心的滤泡性树突状细胞，占所有软组织肉瘤的0.4%。出现伴有支气管内蔓延的肺滤泡性树突状细胞肉瘤以及作为累及纵隔淋巴结的前纵隔肿块极为罕见。我们报告一例34岁不吸烟男性患者，其胸痛3个月。胸部CT检查发现左肺门周围有分叶状肿块，支气管内蔓延至左主支气管并伴有纵隔淋巴结肿大。支气管镜引导下对支气管内肿块进行的冷冻活检结果不明确。经过全面的多学科讨论后，患者接受了左侧肺切除术、纵隔肿块切除术及系统性淋巴结清扫术。采用免疫组织化学进行的组织学检查显示为滤泡性树突状细胞肉瘤。
