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胸膜孤立性纤维瘤

Solitary fibrous tumor of the pleura.

作者信息

Robinson Lary A

机构信息

Thoracic Oncology Program, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL 33612, USA.

出版信息

Cancer Control. 2006 Oct;13(4):264-9. doi: 10.1177/107327480601300403.

DOI:10.1177/107327480601300403
PMID:17075563
Abstract

BACKGROUND

The solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura. Only about 800 cases have been reported in the medical literature. The tumor appears to be unrelated to malignant pleural mesothelioma, the most common primary tumor of the pleura.

METHODS

In just over half of these cases, the neoplasm presents as an asymptomatic mass, is often quite large, and is benign in 78% to 88% of patients. The initial evaluation and diagnosis, tumor classification, surgical treatment, results of therapy, and long-term prognosis are reviewed, based on a selective review of the literature from MEDLINE beginning 1980.

RESULTS

Complete en bloc surgical resection is the preferred treatment of benign and malignant varieties of the tumor. The pedunculated tumors attached to the visceral pleura can be effectively treated with a wedge resection of lung. Sessile tumors arising on the lung require a larger lung resection. Sessile tumors on the chest wall require wide local excision, often with chest wall resection because of their propensity for local recurrence. Adjuvant therapy remains controversial in SFTP.

CONCLUSIONS

Benign SFTP has a high cure rate and an 8% local recurrence rate that is usually amenable to curative re-excision. Malignant SFTP, especially the more common sessile type, has a 63% recurrence rate even with complete resection. The majority of patients with recurrent disease die of the tumor within 2 years. Nevertheless, the overall long-term cure rate for all patients is 88% to 92%.

摘要

背景

胸膜孤立性纤维瘤(SFTP)是一种罕见的原发性肿瘤,起源于间皮衬里胸膜下方乳晕组织中的间充质细胞。医学文献中仅报道了约800例病例。该肿瘤似乎与胸膜最常见的原发性肿瘤恶性胸膜间皮瘤无关。

方法

在这些病例中,略多于一半的患者肿瘤表现为无症状肿块,通常体积较大,78%至88%的患者肿瘤为良性。基于对1980年以来MEDLINE文献的选择性回顾,对初始评估与诊断、肿瘤分类、手术治疗、治疗结果及长期预后进行了综述。

结果

完整的整块手术切除是该肿瘤良性和恶性类型的首选治疗方法。附着于脏层胸膜的带蒂肿瘤可通过肺楔形切除术有效治疗。起源于肺的无蒂肿瘤需要更大范围的肺切除。胸壁上的无蒂肿瘤需要广泛局部切除,由于其局部复发倾向,通常需要切除胸壁。辅助治疗在SFTP中仍存在争议。

结论

良性SFTP治愈率高,局部复发率为8%,通常可通过根治性再次切除治愈。恶性SFTP,尤其是较常见的无蒂型,即使完全切除,复发率仍为63%。大多数复发患者在2年内死于肿瘤。然而,所有患者的总体长期治愈率为88%至92%。

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