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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 / Copyright

    Affiliations: Department of Dermatology, ‘Carol Davila’ University of Medicine and Pharmacy, 050474 Bucharest, Romania, Department of Obstetrics and Gynecology, ‘Carol Davila’ University of Medicine and Pharmacy, 050474 Bucharest, Romania, Department of Endocrinology, ‘Carol Davila’ University of Medicine and Pharmacy, 050474 Bucharest, Romania, Department of Urology, ‘Carol Davila’ University of Medicine and Pharmacy, 050474 Bucharest, Romania, Department of Plastic and Reconstructive Surgery, ‘Carol Davila’ University of Medicine and Pharmacy, 050474 Bucharest, Romania
  • Article Number: 1338
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    Published online on: September 22, 2021
       https://doi.org/10.3892/etm.2021.10773
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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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Copy and paste a formatted citation
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
Copy and paste a formatted citation
x
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
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