
==== Front
Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00715-5
10.1016/j.radcr.2024.07.128
Case Report
Adrenal ganglioneuroma with nodal metastases on 123I-MIBG SPECT/CT and 18F-FDG PET/CT
Tsukamoto Suzune MD suzune.tsukamoto@gmail.com
a⁎
Kawabata Kazuna MD, PhD a
Ito Shunsuke MD a
Ando Saya MD a
Kuriyama Kaori MD a
Morooka Shin MD a
Muramaki Mototsugu MD, PhD b
Kanagaki Mitsunori MD, PhD a
Hosono Makoto MD, PhD c
a Department of Diagnostic Radiology, Hyogo Prefectural Amagasaki General Medical Center, Hyogo, Japan
b Department of Urology, Hyogo Prefectural Amagasaki General Medical Center, Hyogo, Japan
c Department of Radiology, Kindai University Faculty of Medicine, Osaka, Japan
⁎ Corresponding author. suzune.tsukamoto@gmail.com
22 8 2024
11 2024
22 8 2024
19 11 51285132
28 4 2024
20 7 2024
22 7 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Ganglioneuroma is a well-differentiated tumor originating from neural crest cells of the sympathetic nervous system. Although benign, a few cases have been reported that ganglioneuroma can metastasize to other sites. We report a case of adrenal ganglioneuroma with para-aortic nodal metastases with low FDG and MIBG uptake. In order to avoid unnecessary wide excision or aggressive medication, it is important to consider the possibility of ganglioneuroma preoperatively even if with metastases.

Keywords

Adrenal ganglioneuroma
Metastatic ganglioneuroma
Lymph node metastasis
18F-FDG PET/CT
123I-MIBG SPECT/CT
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pmcIntroduction

Ganglioneuroma, ganglioneuroblastoma, and neuroblastoma are tumors of the sympathetic nervous system originating from neural crest and arise wherever sympathetic tissue exists [1]. Ganglioneuroma is the most benign and well-differentiated of these tumors, while ganglioneuroblastoma and neuroblastoma have more aggressive nature. Ganglioneuroma often occurs at the posterior mediastinum (41.5%), retroperitoneum (37.5%) and adrenal gland (21%) [1]. Patients of all ages are affected, predominantly children and young adults [2]. Ganglioneuroma is often discovered as incidentaloma since it is asymptomatic until growing large [3]. Although rare, a few cases have been reported that ganglioneuroma can metastasize to other sites [[4], [5], [6], [7], [8], [9]]. In this case report, we described a 40-year-old male with adrenal ganglioneuroma with nodes metastasis.

Case report

A 40-year-old male underwent routine abdominal ultrasound that showed 90 mm mass in the left retroperitoneal space with no abdominal symptoms. Unenhanced CT revealed a clear and smooth margined, hypodense 95 × 72 mm mass of the left adrenal gland which had lower density compared to psoas muscle except for punctate calcification. In contrast-enhanced CT, it showed slight contrast enhancement. No cystic degeneration was found. (Figs. 1A-C, black arrow: calcification). Endocrinological examination results, including serum cortisol, androgen hormones, and urine levels of catecholamines and their metabolites, were normal. Additionally, CT revealed enlarged para-aortic lymph nodes (Figs. 1D and E, arrowheads) suspicious for nodes metastasis. On MRI, the adrenal lesion showed homogenous hypointensity on T1-weighted imaging with no signal loss on opposed phase excluding adipose component (Fig. 2A and B). It showed heterogeneous hyperintensity on T2-weighted imaging suggesting myxoid component (Fig. 2C). Diffusion-weighted imaging and apparent diffusion coefficient map showed partial diffusion restriction in the adrenal mass (Figs. 2D and E). The para-aortic lymph nodes showed homogenous hypointensity on T2-weighted images and diffusion restriction (Figs. 2G-I, arrowheads). However, 18F-FDG PET/CT demonstrated mild and heterogeneous FDG uptake in the adrenal lesion (Figs. 3A and B, arrows, SUVmax, 3.1) and mild uptake in the lymph nodes (Fig. 3C and D, arrowheads, SUVmax, 2.0), which decreased the likelihood of aggressive tumors such as adrenocortical carcinoma, malignant lymphoma and metastatic tumors [10,11]. As neural crest-derived tumors were considered a differential diagnosis, 123I-MIBG SPECT/CT was performed, which revealed partial uptake in the adrenal lesion (Fig. 3E, arrow) and no uptake in the retroperitoneal lymph nodes (Figs. 3F and G, arrowheads).Fig. 1 CT images of the left adrenal lesion and the para-aortic lymph nodes. Unenhanced CT revealed a clear and smooth margined, hypodense mass of the left adrenal gland (A, white arrow) with punctate calcification (A, black arrow). Contrast-enhanced CT showed slight contrast enhancement in the adrenal mass (B, early phase, C, delayed phase). No cystic degeneration was found. CT also revealed enlarged para-aortic lymph nodes (D, E, delayed phase, arrowheads). A punctate calcification was also identified in one of the nodal lesions (D, black arrow).

Fig 1

Fig. 2 Magnetic resonance images of the left adrenal lesion and the para-aortic lymph nodes. The adrenal lesion exhibited homogenous hypointensity on T1-weighted imaging (A, in phase, B, opposed phase), which showed no signal loss on opposed phase excluding adipose component. On T2-weighted imaging, it showed heterogeneous hyperintensity suggesting myxoid component (C). There was partial diffusion restriction in the adrenal mass (D, diffusion-weighted imaging, E, apparent diffusion coefficient map). The adrenal mass was located between the spleen and the left kidney on T2-weighted imaging in the coronal plane (F, white arrow). The enlarged nodal lesions showed a homogenous hypointensity on T2-weighted imaging (G), and diffusion restriction (H, diffusion-weighted imaging, I, apparent diffusion coefficient map). The images in Fig. 2 (G-I) were captured at the same level as those in Fig. 1 (E).

Fig 2

Fig. 3 18F-FDG PET/CT and 123I-MIBG SPECT/CT of the adrenal lesion and the para-aortic lymph nodes. 18F-FDG PET/CT demonstrated mild and heterogeneous FDG uptake in the adrenal lesion (A, MIP, B, fusion transaxial, arrow; adrenal mass, SUVmax, 3.1) and mild uptake in the lymph nodes (C, D, fusion transaxial, arrowheads; SUVmax, 2.0). 123I-MIBG SPECT/CT revealed partial uptake in the adrenal lesion (E, fusion transaxial, arrow) and no uptake in the retroperitoneal lymph nodes (F, G, fusion transaxial, arrowheads).

Fig 3

The patient underwent open left adrenalectomy and the excision of para-aortic lymph nodes. Histologically, the left adrenal lesion comprised scattered mature ganglion cells and surrounding bundles of Schwann cells, leading the diagnosis of ganglioneuroma (Fig. 4A, HE × 100, 4B, HE × 400, arrowheads; ganglion cells). Within the tumor, there were embedded normal adrenal gland cells (Fig. 4A, arrows), consistent with the prediction that its origin was the left adrenal gland. The lymph nodes also demonstrated typical ganglioneuroma pathology negative for malignancy. The patient had an uneventful postoperative course and was free of recurrence 7 months after surgery so far.Fig. 4 Histological sections of the adrenal ganglioneuroma. The adrenal lesion comprised scattered mature ganglion cells and surrounding bundles of Schwann cells (A, HE × 100, B, HE × 400, arrowheads; ganglion cells). Within the lesion, there was embedded normal adrenal gland cells (A, arrows), consistent with the prediction that its origin was the left adrenal gland.

Fig 4

Discussion

Ganglioneuromas are rare neuroectodermal tumors with benign nature. They appear as well-circumscribed oval, crescentic, or lobulated masses with an attenuation less than that of muscle [2]. They contain calcification in about 20% of cases, mostly discrete and punctate rather than amorphous and coarse as in neuroblastomas [12]. On MRI, their T2 signal intensity depends on the proportion of myxoid stroma to cellular components and the amount of collagen fibers in the tumor and one of the MRI characteristics of ganglioneuroma is curvilinear bands of low signal intensity on T2-weighted images, the so-called whorled appearance [2]. Deflorenne et al. have reported that increased uptake on 18F-FDG PET/CT was identified in 42.2% of cases (median SUVmax: 3.65) [13]. They also showed that 26.7% of the tumors showed increased uptake on 123I-MIBG scintigraphy, for which the percentage of patients with increased uptake was higher (33.3%) in the pediatric group (4 to 15 years old) than in the adult group (over 15 years old), with no clear relationship between 18F-FDG PET and 123I-MIBG scintigraphy uptake [13]. In the present case, the adrenal lesion showed mild FDG and partial MIBG uptakes, which could be consistent with ganglioneuroma. The possible causes for 123I-MIBG uptake in ganglioneuroma include positive endocinological activity [14] and focal nodular hyperplasia [15]. In pediatric patients with peripheral neuroblastic tumors, strong 123I-MIBG uptake indicated unfavorable histopathology with a high mitotic activity [16]. The present case, however, had no sign of active proliferation in the adrenal lesion despite of metastatic potential.

Although ganglioneuromas are usually considered benign, they can metastasize in some rare cases to other sites, such as the lymph nodes [[4], [5], [6], [7]], liver [8,9], spleen [9], and soft tissues [4]. Such metastases are believed to be due to the spreading of neuroblastoma or ganglioneuroblastoma that subsequently matured to ganglioneuroma [17]. In the study of 104 cases of adrenal ganglioneuroma, 6 patients (5.8%) had secondary intra-abdominal lymph-node invasion confirmed by pathology, with a median age of 20 years, no hormonal hypersecretion, and increased MIBG uptake in 1 case (1/4) [13]. Among them, no recurrence or local progression was observed after surgery with a median duration of 45 months [13]. Because ganglioneuroma has excellent prognosis and is unlikely to cause tumor-related death [18] even in cases having metastases [5,7], it is important to consider the possibility of ganglioneuroma preoperatively due to low uptake on 18F-FDG PET/CT and 123I-MIBG scintigraphy in order to avoid unnecessary wide excision or aggressive medication.

Conclusion

This case report described a case of adrenal ganglioneuroma with para-aortic nodal metastases, which highlights the need for considering ganglioneuroma as differential diagnosis of retroperitoneal mass with lymphadenopathy. Low FDG and MIBG accumulation is indicative of ganglioneuroma and may help in making an accurate diagnosis preoperatively, which can avoid unnecessary wide excision or aggressive medication.

Patient consent

Written and informed consent was obtained from the patient.

Competing Interests: The authors declare no conflicts of interest associated with this manuscript.
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