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11年后的血管外皮细胞瘤:一种罕见软组织肉瘤的延迟复发

Hemangiopericytoma 11 years later: delayed recurrence of a rare soft tissue sarcoma.

作者信息

Cheng Keng Peng, Wong Wei-Jin, Hashim Shahrul, Mun Kein Seong

机构信息

Cardiothoracic Surgery Unit, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Department of Pathology, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

出版信息

J Thorac Dis. 2017 Sep;9(9):E752-E756. doi: 10.21037/jtd.2017.08.74.

Abstract

Hemangiopericytomas (HPCs) are uncommon tumours. We present the case of a 41-year-old female with multiple resections at different sites over the course of 11 years. The approach considerations, as well as treatment options and prognosis are discussed. A 41-year-old female with two previous resections for intracranial meningeal HPC in 2004 and 2008, as well as adjuvant radiotherapy, presented in 2015 with left intrathoracic and left hip recurrence confirmed by positron emission tomography/computed tomography (PET/CT). She underwent left proximal femur resection/reconstruction and video-assisted thoracoscopic surgery (VATS) resection of the intrathoracic tumour was attempted. She was discharged home on her 4th post-operative day with minimal pain. There were no neurosensory or motor deficits. Any patient who has been diagnosed with HPC in the past who develops new symptoms should be worked up for recurrence, regardless of the length of disease-free interval, as our case study suggested. There has yet to be a standardized follow-up regime due to the rarity of these tumours. HPC remains a rare soft tissue sarcoma with high recurrence rate. Planned VATS evaluation and resection is possible provided complete resection with clear surgical margins can be achieved, as clear surgical margins offer the best chance of survival.

摘要

血管外皮细胞瘤(HPCs)是一种罕见肿瘤。我们报告了一例41岁女性患者,在11年的病程中于不同部位进行了多次手术切除。文中讨论了手术入路的考量因素、治疗方案及预后。一名41岁女性,曾于2004年和2008年因颅内脑膜HPC接受过两次手术切除,并接受了辅助放疗,2015年因正电子发射断层扫描/计算机断层扫描(PET/CT)证实左胸内及左髋部复发。她接受了左股骨近端切除/重建手术,并尝试通过电视辅助胸腔镜手术(VATS)切除胸内肿瘤。术后第4天她出院回家,疼痛轻微。无神经感觉或运动功能缺损。正如我们的病例研究所示,任何既往诊断为HPC且出现新症状的患者,无论无病间期长短,均应排查复发情况。由于这些肿瘤罕见,目前尚无标准化的随访方案。HPC仍然是一种罕见的软组织肉瘤,复发率高。如果能够实现完整切除且手术切缘清晰,计划进行VATS评估和切除是可行的,因为清晰的手术切缘提供了最佳的生存机会。

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