Gun Feryal, Erginel Basak, Unüvar Aysegul, Kebudi Rejin, Salman Tansu, Celik Alaaddin
Department of Pediatric Surgery, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Pediatr Hematol Oncol. 2012 Mar;29(2):141-7. doi: 10.3109/08880018.2011.646385.
Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.
原发性纵隔恶性肿瘤是罕见肿瘤,可起源于任何纵隔器官或组织,如胸腺、神经源性、淋巴、生殖或间叶组织。作者回顾了25年间诊断的所有原发性小儿纵隔肿块病例,以确定其临床表现模式、组织学类型及手术治疗结果。在本研究中,对1985年至2011年间诊断的120例原发性小儿纵隔肿块病例,根据其年龄、性别、症状、解剖位置、手术治疗及组织病理学评估进行回顾性分析。患者的中位年龄为5.8岁。其中良性肿瘤34例,恶性肿瘤86例。30例患者无症状。患者的常见症状为咳嗽、呼吸困难、乏力、发热、腹痛、背痛及神经症状。根据其起源,表现为神经源性肿瘤(38.3%)、淋巴瘤(18.3%)、未分化肉瘤(15%)、生殖细胞肿瘤(7.5%)以及其他肿瘤(22%),包括胸腺病变、淋巴管瘤、横纹肌肉瘤、脂肪瘤、血管瘤及肾母细胞瘤。86例患者进行了肿瘤完整切除,11例患者进行了肿瘤部分切除。23例患者进行了活检。由于儿童纵隔肿块常表现为无症状或非特异性症状,如上气道疾病,其诊断可能具有挑战性。对于良性和恶性的神经源性肿瘤或淋巴瘤,若有可能完整切除,则建议手术治疗。尽管手术是大多数纵隔肿瘤的主要治疗方法,但仍需要经验丰富的多学科治疗方法。