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Mediastinal myelolipoma/extramedullary hematopoiesis presenting as a mass: rare differential diagnosis among mediastinal tumors

机译:纵隔骨髓脂肪瘤/硬脑膜外造血细胞呈肿块:纵隔肿瘤罕见的鉴别诊断

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摘要

Objectives: Mediastinal myelolipoma/extramedullary hematopoiesis presenting as a mass is infrequent and can lead to misdiagnosis. Here we describe a large series aiming to illustrate the clinicopathologic features. Methods: We retrospectively searched mediastinal tumors and myelolipoma diagnosed at the Department of Pathology, West China Hospital from 2010 to 2015 and collected 14 mediastinal myelolipoma/extramedullary hematopoiesis cases presenting as an encapsulated mass among 1324 mediastinal mass diseases and 252 myelolipomas. Results: There were 8 females and 6 males aged from 35 to 67 years old, most of whom were diagnosed incidentally. Cross-sectional imaging revealed encapsulated masses located in the posterior mediastinum with fat and soft tissue density showing heterogeneous enhancement. Radiologic diagnosis was neurogenic tumor for most cases. All but one patient underwent surgery and postoperative pathologic findings showed fat and hematologic elements. Considering the accompanying hematologic disorders, 5 patients were diagnosed as extramedullary hematopoiesis and the remaining 9 as myelolipoma. The average hematopoietic tissue percentage in extramedullary hematopoiesis was 70%, significantly higher than it was in myelolipoma. Patients showed no sign of recurrence or metastasis apart from the patient with hepatocellular carcinoma. Conclusions: Mediastinal myelolipoma/extramedullary hematopoiesis is a rare entity of solid tumors in the posterior mediastinum, affecting patients from their third decades, with no sex predilection and lacking unique clinical symptoms, and may be misdiagnosed as a malignant tumor on cross-sectional imaging. The final diagnosis relies on pathologic findings, and the precise classification of myelolipoma or extramedullary hematopoiesis relies on percentage of hematopoietic tissue and accompanying clinical symptoms. Surgery is the recommended treatment.
机译:目的:纵隔骨髓脂肪瘤/硬脑膜外造血细胞少见,可导致误诊。在这里,我们描述了一个大系列,旨在说明临床病理特征。方法:回顾性分析2010年至2015年在华西医院病理科诊断出的纵隔肿瘤和骨髓脂肪瘤,收集13例纵隔肿块疾病和252例骨髓脂肪瘤中14例纵隔性骨髓脂肪瘤/髓外造血病例。结果:年龄在35至67岁之间的女性为8位女性,男性为6位,其中大多数被偶然诊断出。横断面成像显示位于纵隔后部的包囊肿块,脂肪和软组织密度显示异质性增强。在大多数情况下,放射学诊断是神经源性肿瘤。除一名患者外,所有患者均接受了手术,术后病理结果显示脂肪和血液学因素。考虑到伴随的血液系统疾病,有5例被诊断为髓外造血,其余9例为骨髓脂肪瘤。髓外造血的平均造血组织百分比为70%,显着高于骨髓性脂肪瘤。除患有肝细胞癌的患者外,患者均未显示复发或转移的迹象。结论:纵隔性骨髓增生异常/硬脑膜外造血是后纵隔实体瘤的一种罕见实体,影响到三十岁以后的患者,无性别偏爱且缺乏独特的临床症状,在横断面成像中可能被误诊为恶性肿瘤。最终诊断取决于病理结果,而骨髓脂肪瘤或髓外造血的精确分类取决于造血组织的百分比和伴随的临床症状。推荐手术治疗。

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