Collet P, Loire R, Guérin J C, Brune J
Rev Pneumol Clin. 1985;41(3):151-5.
Twelve cases of apparently primary thoracic hemangiopericytoma are reported: 7 intrapulmonary and 5 extrapulmonary. These cases, taken together with 75 others already published, serve as a basis to describe the characteristics of thoracic hemangiopericytomas. Intrapulmonary forms raise the problem of a single peripheral X-ray lesion, often detected in a routine film, with no accompanying endoscopic abnormality. Extrapulmonary forms also take the form of a well defined tumor with diaphragmatic or mediastinal parietal connections. Up to the present, the diagnosis has always been made at thoracotomy. It is probable that a preoperative diagnosis will be possible in the future by transbronchial biopsy. Silver stains and the use of the electron microscope form the basis of histological diagnosis. However it is not possible to distinguish by histology between benign and malignant forms, nor between primary or metastatic hemangiopericytomas. It is for this reason that doubt will long persist as to the primary nature of the tumor and only prolonged survival of patients after excision confirms that the lesion was indeed primary. Treatment is essentially surgical. New high energy radiotherapy techniques and new possibilities in chemotherapy (using adriamycin) should improve the prognosis in those forms which run a malignant course. However the standard therapeutic strategy for these rare tumors has yet to be defined.
报告了12例明显为原发性胸内血管外皮细胞瘤的病例：7例为肺内型，5例为肺外型。这些病例与已发表的其他75例病例一起，作为描述胸内血管外皮细胞瘤特征的基础。肺内型表现为单个周围性X线病变的问题，常在常规胸片中发现，且无伴随的内镜异常。肺外型也表现为边界清晰的肿瘤，与膈肌或纵隔壁层相连。到目前为止，诊断一直是在开胸手术时做出的。未来通过经支气管活检有可能进行术前诊断。银染色和电子显微镜的使用是组织学诊断的基础。然而，通过组织学无法区分良性和恶性形式，也无法区分原发性或转移性血管外皮细胞瘤。正是由于这个原因，关于肿瘤的原发性性质的疑问将长期存在，只有在切除后患者长期存活才能证实病变确实是原发性的。治疗主要是手术治疗。新的高能放疗技术和化疗（使用阿霉素）的新可能性应能改善那些呈恶性病程的形式的预后。然而，这些罕见肿瘤的标准治疗策略尚未确定。
