胸部神经节瘤(Thoracic Ganglioneuroma)CT多平面重建病例图片影像诊断分析

周围神经   2010-11-08

 【临床病史】:患者, 7岁小孩近期出现干咳,按肺炎治疗。7-year-old female with nonproductive cough recently treated for pneumonia.

【影像图片】CT图像


胸部神经节瘤

胸部神经节瘤胸部神经节瘤

【影像表现】:Figure 1: Scout view of the chest demonstrates a right chest mass that does not obscure the right heart border consistent with a posterior mediastinal lesion (arrow). 胸部平扫显示一个右胸部肿块,右心缘尚清醒,说明肿块位于后纵隔。

    Figure 2, Figure 3, and Figure 4: Axial and coronal CT images demonstrate a large homogeneous, hypoattenuating, well circumscribed mass (arrows) in the posterior mediastinum that measures 5 cm x 5 cm x 8 cm. It has sharply defined walls and there is no associated enhancement or calcifications. No osseous destructive changes are seen. Mild compressive atelectasis is noted adjacent to the lesion. The remainder of the lung is clear.

    图2-4:轴位和冠状位CT显示后纵隔一个较大的密度均匀的局限性低密度影,大小约5×5×8cm,肿块有边界清楚的薄壁,无明显强化或钙化,无骨质破坏,邻近的肺组织轻度受压不张,余肺尚清晰。

【影像诊断】:Thoracic Ganglioneuroma 胸部神经节瘤

【诊断要点】:神经节瘤是一种少见的良性肿瘤,是由神经节神经母细胞瘤和神经母细胞瘤成熟化而形成,与两者在影像上难以鉴别,但是后两者可以出现转移,而神经节瘤转移很少见;影像表现为密度均匀、边界清楚的局限性肿块,可有钙化; 可见于沿交感神经节和肾上腺的任何部位,多位于后纵隔、腹膜后及肾上腺;可以是分泌性的肿瘤。

【讨论】:Ganglioneuroma is a rare, benign neoplasm of the sympathetic nervous system. It is the result of maturation of a ganglioneuroblastoma or neuroblastoma into a ganglioneuroma and may develop within a neuroblastoma treated with chemotherapy. Metastasis is rare and is thought to be the result of metastatic ganglioneuroblastoma or neuroblastoma that have subsequently matured. Ganglioneuromas are differentiated from ganglioneuroblastomas and neuroblastomas by the presence of mature ganglion cells and the absence of immature elements such as neuroblasts, intermediate cells, or mitotic figures.神经节瘤是一种少见的、良性交感神经系统肿瘤,它是由神经节神经母细胞瘤或神经母细胞瘤成熟转变而来,也可以是由化疗中的神经母细胞瘤发展而来的。转移是少见的,即使有转移,也被认为是来源于成熟前的神经节神经母细胞瘤或神经母细胞瘤。神经节细胞瘤与神经节神经母细胞瘤及神经母细胞瘤的鉴别主要通过是否存在成熟的神经节细胞和是否缺乏幼稚的成分如神经母细胞、中间细胞以及有丝分裂像等。

    Ganglioneuromas are radiologically indistinguishable from ganglioneuroblastomas and neuroblastomas aside from the presence of metastasis which is rare with ganglioneuromas. The imaging characteristics of these tumors are homogenous, well-circumscribed, solid masses. They tend to have low attenuation on CT and may have heterogenous or homogenous enhancement. Calcifications are seen in 42-60% of cases and are usually punctuate as opposed to the coarse pattern more common with ganglioneuroblastomas or neuroblastomas. MR imaging is more effective in evaluating for regional invasion and extension into the epidural space.神经节瘤从放射学上难于与神经节神经母细胞瘤和神经母细胞瘤,除非两者出现转移,相应神经节瘤很少转移。这些肿瘤的影像特征都是密度均匀,边界清楚的实性肿块,CT上多表现为低密度,可能会有均匀或不均匀的强化。钙化见于42-60%的病例,MR在评估病变对邻近区域的侵犯和硬膜下腔的扩展更有帮助。

    Ganglioneuromas can arise anywhere along the paravertebral sympathetic ganglia and adrenal gland. The most common location is the posterior mediastinum (41%), retroperitoneum (37%), adrenal gland (21%), and neck. There are rare reported cases in the tongue, mandible, bladder, uterus, ovary, spermatic cord, testes, prostate, skin and bone. Occasionally, these tumors may secrete catecholamines, testosterone, or vasoactive intestinal polypeptide. Females are affected slightly more often than males with a female-to-male ratio of approximately 1.5:1. All ages can be affected, but the median age at diagnosis is 7 years.神经节瘤可以起源于脊柱旁交感神经节和肾上腺的何部位,最常见的部位是后纵隔(41%),腹膜后腔(37%),肾上腺(21%)以及颈部。也有很少的报道见于舌部、下颌骨、膀胱、子宫、卵巢、精索、睾丸、前列腺、皮肤以及骨骼。偶尔,肿瘤可以分泌儿茶酚胺、睾酮、或血管活性肠肽。女性发病率稍高于男性,男女比约1.5:1,所有的年龄都可以被影响,诊断的平均年龄是7岁。

    Treatment usually involves complete surgical resection and patients have an excellent prognosis. Recurrent tumors are rare.处理通常包括完全外科切除,病人预后良好,复发非常罕见。

(参考来源:,ACR Thursday, May 14, 2009,作者:freemanpyw译)
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