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复发性胸腔内去分化脂肪肉瘤:一例报告及文献复习

Recurrent intrathoracic dedifferentiated liposarcoma: A case report and literature review.

作者信息

Ashraf Umair, Dudekula Rizwan Ahmed, Roy Swathi, Burack Joshua, Malik Sandeep, Khaja Misbahuddin

机构信息

Division of Pulmonary and Critical Care Medicine, Bronx Care Health System, Affiliated with Icahn School of Medicine at Mount Sinai, 1650 Grand Concourse, Bronx, NY 10457, USA.

Department of Medicine, Bronx Care Health System, Affiliated with Icahn School of Medicine at Mount Sinai, 1650 Grand Concourse, Bronx, NY 10457, USA.

出版信息

Respir Med Case Rep. 2019 Feb 21;26:281-284. doi: 10.1016/j.rmcr.2019.02.016. eCollection 2019.

Abstract

BACKGROUND

Liposarcoma (LPS) is the second most common type of soft tissue sarcoma, accounting for approximately 15-20% of all the sarcomas. Primary intrathoracic LPS, however, is quite rare. LPS is a malignant mesenchymal tumor, comprised of lipogenic tissue with varying degrees of atypia. It can be subclassified into well-differentiated LPS (WDLPS), myxoid LPS (MLPS)/round cell LPS, pleomorphic LPS (PLPs), and dedifferentiated LPS (DDLPS), based on the histology.

CASE PRESENTATION

A 76-year-old male patient initially presented to the emergency room with a complaint of precordial chest pain for one month. Computed tomography (CT) of his chest showed a large, 8 cm × 8 cm x 10 cm, supradiaphragmatic, complex solid mass in the lower left hemithorax, along the anterior chest wall. Chest wall mass excision revealed dedifferentiated LPS, with excision of margins. Positron emission tomography (PET) scan did not show metastatic disease. Seven months later, he presented with shortness of breath, and CT of the chest showed large, left pleural-based masses, causing compression of surrounding structures. He was not a candidate for surgical resection. This patient subsequently failed chemotherapy and opted for hospice.

CONCLUSION

Intrathoracic LPS is a rare tumor. Recurrence is higher with dedifferentiated histology forms. Radical surgery with excision of margins is the primary recommended treatment.

摘要

背景

脂肪肉瘤(LPS)是第二常见的软组织肉瘤类型,约占所有肉瘤的15%-20%。然而,原发性胸内脂肪肉瘤相当罕见。脂肪肉瘤是一种恶性间充质肿瘤,由具有不同程度异型性的脂肪生成组织组成。根据组织学,它可分为高分化脂肪肉瘤(WDLPS)、黏液样脂肪肉瘤(MLPS)/圆形细胞脂肪肉瘤、多形性脂肪肉瘤(PLPs)和去分化脂肪肉瘤(DDLPS)。

病例介绍

一名76岁男性患者最初因心前区胸痛1个月就诊于急诊室。胸部计算机断层扫描(CT)显示左半胸下部、前胸壁处有一个8厘米×8厘米×10厘米的巨大膈上复杂实性肿块。胸壁肿块切除显示为去分化脂肪肉瘤,并切除了切缘。正电子发射断层扫描(PET)未显示转移性疾病。7个月后,他出现呼吸急促,胸部CT显示左胸膜处有巨大肿块,压迫周围结构。他不适合手术切除。该患者随后化疗失败,选择了临终关怀。

结论

胸内脂肪肉瘤是一种罕见肿瘤。去分化组织学类型的复发率更高。推荐的主要治疗方法是切除切缘的根治性手术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e56/6389794/45c165d4dba5/gr1.jpg

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