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Giant adrenal ganglioneuroma in children: a case report

BACKGROUND: Ganglioneuromas (GNs) arise from the Schwann cells, ganglion cells, and neuronal tissues, and are extremely rare, slow-growing, benign tumors. GN has usually grown very large when it is diagnosed since no specific clinical symptoms or laboratory findings indicating GN are available, espe...

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Detalles Bibliográficos
Autores principales: Hu, Mingqiu, Tang, Zhizhong, Cai, Yong, Yuan, Xiaolu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9568632/
https://www.ncbi.nlm.nih.gov/pubmed/36239827
http://dx.doi.org/10.1007/s12672-022-00573-3
Descripción
Sumario:BACKGROUND: Ganglioneuromas (GNs) arise from the Schwann cells, ganglion cells, and neuronal tissues, and are extremely rare, slow-growing, benign tumors. GN has usually grown very large when it is diagnosed since no specific clinical symptoms or laboratory findings indicating GN are available, especially when it occurs in the retroperitoneal space. Total resection of the tumor is the recommended treatment. CASE SUMMARY: We present the imaging and pathological findings of a giant adrenal GN in a child. A 7-year-old boy suffered from nausea and postprandial vomiting for 1 week with no precipitating factors. There was no family history of any disease, and the boy did not suffer from any disease in the past. Biochemical examination showed normal results. Physical examination showed an immobilized palpable mass in the left abdominal area. Abdominal computed tomography revealed a 13 cm × 10 cm solid mass in the retroperitoneal space. The mass showed slight and heterogeneous enhancement after injection of a contrasting agent. The mass was surgically resected locally to address the embedded abdominal vessels, and the histopathological and immunohistochemical diagnosis of the mass was GN. After the surgery, the symptoms of nausea and vomiting were relieved, and no complications occurred. CONCLUSION: GN should be considered when a child presents with a giant retroperitoneal hypodense mass and the mass presents uneven and delayed enhancement. Histopathology is the golden standard for the diagnosis of GN. Currently, surgical excision is the optimal treatment.