Computed tomography characteristics of adrenal ganglioneuroma: a retrospective analysis of 30 pathologically-confirmed cases

Shao, Meihua; Zhang, Wenming; Niu, Zhiwei; Chen, Songkuan; Jia, Yuzhu; An, Yongyu; Zhu, Fu‐Gang; Wang, Jian

doi:10.1177/0300060520945510

Cited by 3 publications

(1 citation statement)

References 25 publications

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“…At imaging evaluation, a cutoff of 3 cm has been suggested between large and small adrenal ganglioneuroma with respect to different behaviors (14). High uptake of I 123 -MIBG (metaiodobenzylguanidine) is associated with a poor prognosis as seen in neuroblastomas and ganglioneuromas with increased mitotic index; while some tumors, regardless of whether they are ganglioneuromas or neuroblastomas, are I 123 -MIBG-negative.…”

Section: Presentation Of An Adrenal Ganglioneuromamentioning

confidence: 99%

Adrenal ganglioneuroma: Prognostic factors (Review)

et al. 2021

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Ganglioneuroma, a rare neural crest-derived tumor, exhibits a benign profile in contrast to other neuroblastic tumors (neuroblastoma/ganglioneuroblastoma). Ganglioneuromas can be found anywhere autonomic ganglia are located, mostly abdominal/pelvic sites followed by the adrenal glands (one-third of cases), mediastinum/thorax and cervical area. Affecting especially children more than 10 years of age, Ganglioneuroma is either asymptomatic or may cause local compressive effects; rarely inducing nonspecific abdominal complains or arterial hypertension related to oversecretion of epinephrine/norepinephrine/dopamine. Despite a good prognosis, adrenalectomy is necessary in order to rule out a malignancy. Open procedure represents the standard therapeutic option; alternatively, centers with large laparoscopic pediatric experience and good stratification protocols have reported successful procedures. High uptake of I 123 -MIBG is associated with a more severe outcome in cases with increased mitotic index. In neuroblastic tumors, neuron-specific enolase >33 ng/ml, age at diagnosis <49 months, and blood vessel invasion indicate a poor prognosis. Concurrent extra-adrenal/adrenal ganglioneuroma is associated with a more severe prognosis; post-surgical complications are more frequent in non-adrenal vs. adrenal ganglioneuroma. Exceptionally, immune-mediated paraneoplastic neurologic syndromes have been reported: anti-N-methyl-D-aspartate receptor encephalitis and opsoclonus-myoclonus-ataxia syndrome. ROHHAD syndrome is the underlying cause in 40-56% of cases of neuroendocrine tumors including ganglioneuroma; 70% of tumors are diagnosed within the first 24 months after hypothalamic obesity onset, associated with a severe prognosis due to hypoventilation, sleep apnea, and dysautonomia. Recently, the PKB/AKT/mTOR/S6 pathway was identified as a tumorigenic pathway in pediatric ganglioneuroma, not in neuroblastoma; mTOR inhibitors are a potential option for pre-operatory tumor shrinkage. Pediatric adrenal ganglioneuroma has a good prognosis if adequately treated; its recognition requires adrenalectomy. Further development of specific biomarkers is needed. In the present article, we aimed to introduce a review of the literature involving adrenal ganglioneuroma based on a practical, multidisciplinary perspective of prognostic factors.

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Section: Presentation Of An Adrenal Ganglioneuromamentioning

confidence: 99%

Adrenal ganglioneuroma: Prognostic factors (Review)

et al. 2021

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Primary adrenal tuberculosis infection in patients with Behcet’s disease presenting as isolated adrenal metastasis by 18F-FDG PET/CT: a rare case report and literature review

Teng¹,

Fan²,

Wang³

et al. 2021

Gland Surg

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Primary adrenal tuberculosis (TB) is a rare type of extrapulmonary tuberculosis (EPTB).A pathological biopsy is usually required to make a definite diagnosis due to nonspecific symptoms.Antituberculous chemotherapy is the main treatment regimen, and cortisol replacement therapy should be added when adrenal insufficiency is involved. Here, we present a 59-year-old man who had recurrence of oral and genital aphthosis for 3 years and was diagnosed with Behcet's disease (BD), which was cured by thalidomide. After 10 days of admission, the patient had sudden abdominal pain in the right upper quadrant with high fever and was diagnosed with acute cholecystitis attack, which was treated by percutaneous transhepatic gallbladder drainage (PTGBD). Further contrast-enhanced CT showed a right adrenal mass with a diameter of 2.0 cm, and PET-CT indicated intense 18 F-fluorodeoxyglucose ( 18 F-FDG) uptake in the right adrenal mass with a maximum standardized uptake value (SUV max ) of 15.2. As a metastatic adrenal mass was suspected, the patient underwent retroperitoneal laparoscopic adrenalectomy. Histopathological and immunohistochemical analysis revealed primary adrenal TB. After routine anti-tuberculosis treatment with isoniazid, rifampin, pyrazinamide and ethambutol for six months, the patient was cured and discharged. In summary, primary unilateral adrenal TB without adrenal insufficiency is difficult to diagnose only on the basis of clinical manifestations and examinations. Further studies are needed to develop an easier and more accurate diagnostic examination.

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Giant ganglioneuroma of the mediastinum: a case report

Song,

Zhang,

Tian

et al. 2024

Front. Oncol.

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Ganglioneuroma (GN) is a rare benign neurogenic tumor that originates from the sympathetic nerves. It is extremely uncommon to find a lesion originating from the mediastinum that occupies the entire left hemithorax. In this report, we present the case of a 48-year-old female patient with a large mediastinal GN who presented with cough, sputum, and wheezing. Multislice spiral-enhanced CT and magnetic resonance imaging (MRI) revealed a large oval mass in the left thoracic cavity. The surgical operation completely resected the lesion, and the histopathological examination of the resected specimen confirmed the diagnosis of giant ganglion cell neuroma of the mediastinum. Due to the low incidence of GN and the lack of specific imaging manifestations, many radiologists may lack sufficient knowledge of GN and may be prone to misdiagnosis, resulting in delayed treatment. To enhance radiologists’ awareness of giant ganglion cell neuroma of mediastinal origin occupying the thoracic cavity, we provided detailed CT/MRI imaging information for this case, along with a brief summary of similar previously reported cases, to highlight the specific clinical and radiological features of this condition.

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Computed tomography characteristics of adrenal ganglioneuroma: a retrospective analysis of 30 pathologically-confirmed cases

Cited by 3 publications

References 25 publications

Adrenal ganglioneuroma: Prognostic factors (Review)

Adrenal ganglioneuroma: Prognostic factors (Review)

Primary adrenal tuberculosis infection in patients with Behcet’s disease presenting as isolated adrenal metastasis by 18F-FDG PET/CT: a rare case report and literature review

Giant ganglioneuroma of the mediastinum: a case report

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