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目的:探讨肾上腺尤文肉瘤临床、病理特点,提高该病的诊治水平。方法:结合文献复习报道我院收治的1例肾上腺尤文肉瘤患者。结果:本例患者行开放剖腹探查:肾上腺肿瘤切除术+血肿清除术,术中见肿块无边界,破裂出血,周围粘连,清除400ml血凝块及腐烂组织一堆。术区活动性出血,凡士林纱布填塞,术后7d取出填塞物,伤口愈合良好出院。病理及免疫组化证实为肾上腺肿物尤文肉瘤,FISH:检测出ESWR1基因易位。结论:肾上腺尤文肉瘤的特点是病史较短,病势凶险,肿瘤具有浸润性,常表现为局部复发和远处转移,病死率高。手术联合化疗、放疗等综合治疗可以提高生存率,但初诊时即有转移、肿瘤巨大、坏死广泛、位于中轴部和初期化疗效果不佳的患者,预后差。
Objective: To investigate the clinical and pathological characteristics of adrenal Ewing’s sarcoma and to improve the diagnosis and treatment of the disease. Methods: One case of adrenal Ewing’s sarcoma admitted to our hospital was reviewed with literature review. Results: In this case, open laparotomy was performed. Adrenal tumor resection + hematoma removal was performed. No intramural tumor, rupture of hemorrhage and adhesion around the tumor were found in the operation, and 400ml blood clots and decay tissue were removed. Active bleeding area, Vaseline gauze packing, remove the stuffing 7d after surgery, the wound healing well discharged. Pathology and immunohistochemistry confirmed adrenal eosinophilic sarcoma, FISH: detection of ESWR1 gene translocation. Conclusion: Adrenal Ewing’s sarcoma is characterized by a short history, dangerous situation, the tumor is invasive, often manifested as local recurrence and distant metastasis, high mortality. Surgery combined with chemotherapy, radiotherapy and other comprehensive treatment can improve the survival rate, but when the newly diagnosed metastasis, tumor huge, extensive necrosis, located in the central axis and the initial chemotherapy in poor patients with poor prognosis.