Pacella Giulia, Brunese Maria Chiara, Donnarumma Federico, Barrassi Michele, Bellifemine Fabio, Sciaudone Guido, Vallone Gianfranco, Guerra Germano, Sallustio Giuseppina
Department of Medicine and Health Sciences "Vincenzo Tiberio", University of Molise, 86100 Campobasso, Italy.
Fondazione Potito, 86100 Campobasso, Italy.
Diagnostics (Basel). 2023 Jun 27;13(13):2190. doi: 10.3390/diagnostics13132190.
Retroperitoneal ganglioneuroma is a rare neuroectodermal tumor with a benign nature. We performed a literature review among 338 studies. We included 9 studies, whose patients underwent CT and/or MRI to characterize a retroperitoneal mass, which was confirmed to be a ganglioneuroma by histologic exam. The most common features of ganglioneuroma are considered to be a solid nature, oval/lobulated shape, and regular margins. The ganglioneuroma shows a progressive late enhancement on CT. On MRI it appears as a hypointense mass in T1W images and with a heterogeneous high-intensity in T2W. The MRI-"whorled sign" is described in the reviewed studies in about 80% of patients. The MRI characterization of a primitive retroperitoneal cystic mass should not exclude a cystic evolution from solid masses, and in the case of paravertebral location, the differential diagnosis algorithm should include the hypothesis of ganglioneuroma. In our case, the MRI features could have oriented towards a neurogenic nature, however, the predominantly cystic-fluid aspect and the considerable longitudinal non-invasive extension between retroperitoneal structures, misled us to a lymphatic malformation. In the literature, it is reported that the cystic presentation can be due to a degeneration of a well-known solid form while maintaining a benign character: the distinguishing malignity character is the revelation of immature cells on histological examination.
腹膜后神经节细胞瘤是一种罕见的良性神经外胚层肿瘤。我们对338项研究进行了文献综述。我们纳入了9项研究，这些研究中的患者接受了CT和/或MRI检查以对腹膜后肿块进行特征性描述，该肿块经组织学检查证实为神经节细胞瘤。神经节细胞瘤最常见的特征被认为是实性、椭圆形/分叶状以及边界规则。神经节细胞瘤在CT上表现为渐进性延迟强化。在MRI上，它在T1加权图像上表现为低信号肿块，在T2加权图像上表现为不均匀高信号。在综述的研究中，约80%的患者描述有MRI“漩涡征”。原始腹膜后囊性肿块的MRI特征不应排除实性肿块的囊性演变，并且在椎旁位置的情况下，鉴别诊断算法应包括神经节细胞瘤的可能性。在我们的病例中，MRI特征可能提示为神经源性性质，然而，主要为囊液成分以及腹膜后结构之间相当大的纵向无侵袭性延伸，使我们误诊为淋巴管畸形。在文献中，据报道囊性表现可能是由于已知实性形式的退变同时保持良性特征：区分恶性特征是组织学检查中出现未成熟细胞。
