Agrawal D, Lahiri T K, Lakhotia S, Singh Deepak
Indian J Chest Dis Allied Sci. 2014 Jul-Sep;56(3):179-82.
Haemangiopericytoma is a rare soft tissue tumour characterised by tightly packed tumour cells situated around thin walled endothelial lined vascular channels, ranging from capillary sized vessels to large gaping sinusoidal spaces. The tumour cells are surrounded by reticulin and are negative for muscle, nerve and epithelial markers. The diagnosis of extra-pulmonary intra-thoracic, extra-pleural mediastinal mass is difficult. It constitutes only 6% of all primary tumours and cysts of the mediastinum. We report the rare occurrence of primary intra-thoracic, extra-pulmonary mediastinal haemangiopericytoma of mesenchymal origin with perivascular localisation. The patient underwent right postero-lateral thoracotomy and post-operatively received chemotherapy with adriamycin (60 mg/m2) on day 1 and ifosfamide (1.5 g/m2) on day 1 to 3. Thirty-seven months after the operation, the patient has been well with evidence of a single recurrence in the left lower lobe.
血管外皮细胞瘤是一种罕见的软组织肿瘤，其特征是紧密排列的肿瘤细胞围绕着薄壁的内皮衬里血管通道，范围从毛细血管大小的血管到巨大的窦状间隙。肿瘤细胞被网状纤维包围，且对肌肉、神经和上皮标志物呈阴性。诊断胸内肺外、胸膜外纵隔肿块很困难。它仅占所有纵隔原发性肿瘤和囊肿的6%。我们报告了罕见的起源于间充质的原发性胸内肺外纵隔血管外皮细胞瘤伴血管周围定位。患者接受了右后外侧开胸手术，术后在第1天接受阿霉素（60mg/m²）化疗，在第1至3天接受异环磷酰胺（1.5g/m²）化疗。术后37个月，患者情况良好，左下叶有单次复发迹象。
