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目的探讨结节型节细胞神经母细胞瘤(GNBn)的CT表现特征。方法回顾性分析经手术、病理证实的10例GNBn的CT及病理学表现。结果 10例中9例为单发病灶,其中5例位于肾上腺,2例位于腹膜后,另3例分别位于纵隔、颈部、盆腔。10例中5例可见淋巴结转移,分别转移至腹膜后(2例)、主动脉旁(1例)、肝门部(1例)、两侧颈动脉鞘区(1例)。CT平扫肿瘤多为单个巨大分叶状低密度肿块,有完整的包膜或假包膜,内密度不均,可见散在或聚集状钙化灶。增强扫描多为不均匀强化,动脉期瘤体实性成分强化明显,静脉期实性成分持续强化,部分病例瘤周或瘤体内可见呈簇状或线状排列的血管影。10例GNBn的共同组织病理特点为以出血性/坏死性神经母细胞瘤结节为特征,同时可看到周围施万基质组织中的少量混合型节细胞神经母细胞瘤或节细胞神经瘤成分。免疫表型:S-100蛋白、CgA、Syn、NSE阳性,CD99阴性。结论 GNBn是一种神经嵴来源的胚胎性恶性肿瘤,其发病年龄、发病部位特异。CT平扫多密度不均,伴特征性钙化灶,增强扫描多为不均匀强化。确诊有赖于病理,免疫组织化学分型更有利于鉴别诊断。
Objective To investigate the CT features of nodular ganglioneuroblastoma (GNBn). Methods The CT and pathological findings of 10 cases of GNBn confirmed by surgery and pathology were retrospectively analyzed. Results Of the 10 cases, 9 were single lesions, of which 5 were in the adrenal gland, 2 in the retroperitoneum and the other 3 in the mediastinum, neck and pelvis. Lymphatic metastasis was seen in 5 of 10 cases, which were transferred to the retroperitoneum (2 cases), aortic (1 case), hilar (1 case), and carotid sheath on both sides (1 case). CT scan of the tumor mostly single large lobulated low-density mass, a complete capsule or pseudocapsule, uneven density, showing scattered or aggregated calcification. Most of the contrast-enhanced MRI were non-uniform enhancement. The solid components of the arterial phase enhanced obviously, and the solid components in the vein continued to be strengthened. In some cases, blood vessels were clustered or linearly arranged in the tumor or tumor. The common histopathological features of 10 cases of GNBn are characterized by hemorrhagic / necrotic neuroblastoma nodules and a small amount of mixed gangliocytic neuroblastoma or ganglioneurocytoma around Schwannoma tissue . Immunophenotype: S-100 protein, CgA, Syn, NSE positive, CD99 negative. Conclusion GNBn is a kind of embryonic malignant tumor derived from neural crest. The age and location of GNBn are specific. CT scan multi-density uneven, with characteristic calcification, enhanced scan mostly uneven enhancement. Diagnosis depends on the pathology, immunohistochemical typing is more conducive to the differential diagnosis.