Macchiarini Paolo, Ostertag Helmut
Hannover Medical School and Department of Thoracic and Vascular Surgery, Heidehaus Hospital, Hannover, Germany.
Lancet Oncol. 2004 Feb;5(2):107-18. doi: 10.1016/S1470-2045(04)01385-3.
The mediastinum is a unique anatomic area containing several structures and pluripotent cells that allow for the development of a range of tumours. Uncommon neoplasms of the mediastinum account for less than 10% of all mediastinal masses and include primary thymic carcinomas, neuroendocrine carcinomas, germ-cell tumours (GCTs), lymphomas, and neurogenic, endocrine, and mesenchymal tumours. Primary thymic carcinomas and neuroendocrine carcinomas, although rare, are highly malignant lesions. GCTs are thought to derive from primitive germ cells and can be classified in seminomatous and non-seminomatous GCTs. They are located predominantly in the anterior mediastinum, as are the primary mediastinal lymphomas that include Hodgkin lymphoma, large B cell lymphoma, and lymphoblastic lymphoma. Neurogenic tumours may arise from peripheral nerves, sympathetic ganglia, or rarely parasympathetic ganglia and are located predominantly in the posterior mediastinum. Endocrine tumours are the ectopic thyroid and parathyroid tumours. Mesenchymal tumours are rare tumours and no less problematic than they are in peripheral sites. The clinical, radiological, and therapeutic aspects of these tumours are reviewed.
纵隔是一个独特的解剖区域，包含多种结构和多能细胞，可引发多种肿瘤。纵隔罕见肿瘤占所有纵隔肿块的比例不到10%，包括原发性胸腺癌、神经内分泌癌、生殖细胞肿瘤（GCT）、淋巴瘤以及神经源性、内分泌性和间叶性肿瘤。原发性胸腺癌和神经内分泌癌虽然罕见，但却是高度恶性的病变。GCT被认为起源于原始生殖细胞，可分为精原细胞瘤性和非精原细胞瘤性GCT。它们主要位于前纵隔，原发性纵隔淋巴瘤（包括霍奇金淋巴瘤、大B细胞淋巴瘤和淋巴母细胞淋巴瘤）也是如此。神经源性肿瘤可能起源于外周神经、交感神经节，很少起源于副交感神经节，主要位于后纵隔。内分泌肿瘤是异位甲状腺和甲状旁腺肿瘤。间叶性肿瘤较为罕见，与外周部位的间叶性肿瘤一样棘手。本文对这些肿瘤的临床、影像学和治疗方面进行了综述。
