Retroperitoneal ganglioneuroma in children: CT and MRI features with histologic correlation

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Abstract

Objective

To demonstrate the CT and MRI features with histologic correlation of retroperitoneal ganglioneuromas in children.

Methods

The diagnostic images (seventeen CT scans and five MR scans) in 17 children with retroperitoneal ganglioneuroma confirmed by operation and histopathology were retrospectively reviewed, and correlated to the histologic findings.

Results

All tumors presented as an oval-shaped, well-defined mass on both CT and MR images. On unenhanced CT images, calcification was detected in six masses (35.3%), and predominantly low attenuation with the CT value ranged from 22 to 38 HU (mean 29.5 HU) in all the tumors. The tumors with CT value less than 30 HU had a relatively larger amount of myxoid stroma on histopathologic sections than those with CT value more than 30 HU. Tumors showed homogeneous low signal intensity on T1-weighted images and inhomogeneous high signal intensity with interlaced or nodular low signal intensity on T2-weighted images. The post-contrast enhancement on both CT and MR images was lacking or slight in early phase, but moderate or marked in late phase. The inhomogeneous high signal intensity on T2WI, as well as the delayed enhancement corresponded to a large amount of myxoid stroma and a relatively small number of cellular components in tumors.

Conclusion

An oval shape, well-defined margin, low attenuation on CT, inhomogeneous hyperintensity on T2WI, and delayed moderate or marked enhancement are typical features of retroperitoneal ganglioneuroma in children. The imaging features correlated well to the histologic findings.

Introduction

Ganglioneuromas originate from neural crest cells that make up the sympathetic ganglia along the spinal column and adrenal medulla. These tumors represent the benign end of the spectrum for the ganglion cell lineage, whereas ganglioneuroblastomas and neuroblastomas comprise the malignant end of this spectrum [1]. In children, retroperitoneal ganglioneuromas are relatively rare and a diagnosis is often not considered when a mass is found in adrenal gland or paravertebral spaces. In addition, a small number of ganglioneuromas may undergo malignant transformation [2], [3]. So, early detection leading to a complete resection of the tumor is clinically important. CT and MR imaging have been found to be useful in detection and diagnosis of ganglioneuroma [4], [5], [6], [7], [8], [9], [10]. To our knowledge, however, there have been only a few case reports or small sample size of studies describing CT and MR imaging of retroperitoneal ganglioneuroma in the pediatric population [11], [12]. In this study, we reviewed a relatively large number of children cases with retroperitoneal ganglioneumoma, and to determine the CT and MRI features with histologic correlation of this tumor.

Section snippets

Materials and methods

The CT and MR images of 17 patients with retroperitoneal ganglioneuroma were retrospectively reviewed. The diagnosis was confirmed by operation and histopathology in all cases, in which 11 tumors arose in adrenal gland and 6 in extra-adrenal retroperitoneum. The 17 patients were 10 males and 7 females, with the age at diagnosis ranged from 3 to 16 years (mean 8.4 years) (Table 1.). Four patients were found with initial symptoms and signs, including diarrhea (n = 1), hypertension (n = 1), and a

Morphologic features

All the tumors occurred in the adrenal gland or extra-adrenal retroperitoneum, and one tumor grew upward along the paravertebral sympathetic nerve chain to the postmediastinum. The largest diameter of the tumors ranged from 48 to 85 mm (mean 61 mm). All the tumors had an oval shape, and were encapsulated and well circumscribed (Fig. 1, Fig. 2, Fig. 3, Fig. 4). No evidence of local invasion was found. In 4 cases, the vena cava and/or trunk were displaced to varying degrees, whereas no vessel was

Discussion

There is a broad spectrum of neurogenic tumors that involve the retroperitoneal space. These tumors can be classified as 3 types: ganglion cell origin, paraganglionic system origin, and nerve sheath origin. Tumors of ganglion cell origin, which include neuroblastoma, ganglioneuroblastoma and ganglioneuroma, often originate in the adrenal gland or in the paravertebral sympathetic plexus. Neuroblastomas and ganglioneuroblastomas are malignant in nature and most often occur in infants and

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1

Both authors contributed equally to this work and should be considered co-first authors.

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