Adrenal ganglioneuroma: Prognostic factors (Review)

  • Authors:
    • Florica Sandru
    • Mihai Cristian Dumitrascu
    • Aida Petca
    • Mara Carsote
    • Razvan-Cosmin Petca
    • Ana Maria Oproiu
    • Adina Ghemigian
  • View Affiliations

  • Published online on: September 22, 2021     https://doi.org/10.3892/etm.2021.10773
  • Article Number: 1338
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Abstract

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 I123‑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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November-2021
Volume 22 Issue 5

Print ISSN: 1792-0981
Online ISSN:1792-1015

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Spandidos Publications style
Sandru F, Dumitrascu MC, Petca A, Carsote M, Petca R, Oproiu AM and Ghemigian A: Adrenal ganglioneuroma: Prognostic factors (Review). Exp Ther Med 22: 1338, 2021
APA
Sandru, F., Dumitrascu, M.C., Petca, A., Carsote, M., Petca, R., Oproiu, A.M., & Ghemigian, A. (2021). Adrenal ganglioneuroma: Prognostic factors (Review). Experimental and Therapeutic Medicine, 22, 1338. https://doi.org/10.3892/etm.2021.10773
MLA
Sandru, F., Dumitrascu, M. C., Petca, A., Carsote, M., Petca, R., Oproiu, A. M., Ghemigian, A."Adrenal ganglioneuroma: Prognostic factors (Review)". Experimental and Therapeutic Medicine 22.5 (2021): 1338.
Chicago
Sandru, F., Dumitrascu, M. C., Petca, A., Carsote, M., Petca, R., Oproiu, A. M., Ghemigian, A."Adrenal ganglioneuroma: Prognostic factors (Review)". Experimental and Therapeutic Medicine 22, no. 5 (2021): 1338. https://doi.org/10.3892/etm.2021.10773