Yang Pei-Yi, Ma Xiao-Li, Zhao Wen, Fu Li-Bing, Zhang Rui, Zeng Qi, Qin Hong, Yu Tong, Su Yan
Department of Medical Oncology, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center of Children's Health, Beijing 100045, China.
World J Clin Cases. 2022 Jul 16;10(20):7116-7123. doi: 10.12998/wjcc.v10.i20.7116. Epub 2022 May 16.
Germ cell tumors (GCTs) account for 2% of human malignancies but are the most common malignant tumors among males aged 15-35. Since 1983, an association between mediastinal GCT (MGCT) and hematologic malignancies has been recognized.
We report a case in which malignant histiocytosis was associated with mediastinal GCTs. The clinical data of a male patient with MGCT admitted to Beijing Children's Hospital were collected retrospectively. The patient was first diagnosed according to imaging and pathological features as having MGCT, and was treated with surgery and chemotherapy. One year after stopping chemotherapy, imaging showed metastases in the right supraclavicular, mediastinum, hilar region and retroperitoneal lymph node, right pleura, right lung, and right para-cardiac margin. Pathological diagnosis of the liver nodular and hilar lymph nodes included systemic juvenile xanthogranuloma and Rosai-Dorfman lesions with malignant transformation ( morphological characteristics and immunophenotype of histiocytic sarcoma). Following diagnosis, the patient accepted chemotherapy with vindesine, cytarabine and dexamethasone. Positron emission tomography-computed tomography showed partial remission. The patient was followed-up for 10 mo after the diagnosis of malignant histiocytosis, and no sign of progression or relapse was observed.
Physicians should recognize the possibility of hematologic malignancies being associated with MGCT. Suitable sites should be selected for pathological examination.
生殖细胞肿瘤（GCTs）占人类恶性肿瘤的2%，却是15 - 35岁男性中最常见的恶性肿瘤。自1983年以来，纵隔生殖细胞肿瘤（MGCT）与血液系统恶性肿瘤之间的关联已被认识。
我们报告一例恶性组织细胞增多症与纵隔生殖细胞肿瘤相关的病例。回顾性收集了一名入住北京儿童医院的纵隔生殖细胞肿瘤男性患者的临床资料。该患者最初根据影像学和病理特征被诊断为纵隔生殖细胞肿瘤，并接受了手术和化疗。化疗结束一年后，影像学检查显示右锁骨上、纵隔、肺门区及腹膜后淋巴结、右侧胸膜、右肺及右心旁缘有转移。肝脏结节及肺门淋巴结的病理诊断包括系统性幼年性黄色肉芽肿和伴有恶性转化的罗萨伊 - 多夫曼病变（组织细胞肉瘤的形态学特征和免疫表型）。确诊后，患者接受了长春地辛、阿糖胞苷和地塞米松化疗。正电子发射断层扫描 - 计算机断层扫描显示部分缓解。在诊断为恶性组织细胞增多症后对患者进行了10个月的随访，未观察到进展或复发迹象。
医生应认识到血液系统恶性肿瘤与纵隔生殖细胞肿瘤相关的可能性。应选择合适部位进行病理检查。
