Total resection of a giant retroperitoneal and mediastinal ganglioneuroma—case report and systematic review of the literature

Abstract

Background Ganglioneuromas (GNs) are extremely rare, slowly growing, benign tumors that can arise from Schwann cells, ganglion cells, and neuronal or fibrous tissues. Due to their origin from the sympathetic neural crest, they show neuroendocrine potential; however, most are reported to be hormonally inactive. Nevertheless, complete surgical removal is recommended for symptom control or for the prevention of potential malignant degeneration. Case Report A 30-year-old female was referred to our oncologic center due to a giant retroperitoneal and mediastinal mass detected in computed tomography (CT) scans. The initial symptoms were transient nausea, diarrhea, and crampy abdominal pain. There was a positive family history including 5 first- and second-degree relatives. Presurgical biopsy revealed a benign ganglioneuroma. Total resection (TR) of a 35 × 25 × 25 cm, 2550-g tumor was obtained successfully via laparotomy combined with thoracotomy and partial incision of the diaphragm. Histopathological analysis confirmed the diagnosis. Surgically challenging aspects were the bilateral tumor invasion from the retroperitoneum into the mediastinum through the aortic hiatus with the need of a bilateral 2-cavity procedure, as well as the tumor-related displacement of the abdominal aorta, the mesenteric vessels, and the inferior vena cava. Due to their anatomic course through the tumor mass, the lumbar aortic vessels needed to be partially resected. Postoperative functioning was excellent without any sign of neurologic deficit. Conclusion Here, we present the largest case of a TR of a GN with retroperitoneal and mediastinal expansion. On review of the literature, this is the largest reported GN resected and was performed safely. Additionally, we present the first systematic literature review for large GN (> 10 cm) as well as for resected tumors growing from the abdominal cavity into the thoracic cavity.

Ganglioneuroma on fine needle aspiration cytology: Case series and review of the literature

“…1 In past cases, inflammatory and tumor marker tests returned normal results. 4,10,11 Retroperitoneal ganglioneuromas secrete enzymes such as catecholamine in only 39% of cases, making laboratory detection in the remaining 61% of cases near impossible. 10,12 When catecholamines are produced by the tumor, they can be detected with urine testing.…”

“…4,10,11 Retroperitoneal ganglioneuromas secrete enzymes such as catecholamine in only 39% of cases, making laboratory detection in the remaining 61% of cases near impossible. 10,12 When catecholamines are produced by the tumor, they can be detected with urine testing. Catecholamine production also causes symptoms such as vomiting and diarrhea, allowing for easier detection.…”

Retroperitoneal ganglioneuroma presenting as an obstructive pyelonephritis: a case report

“…First described by Loretz in 1870, GN is mostly seen in pediatric populations, with 60% of cases before 20 years [ 8 ]. The median age of the diagnosis is ~7 years [ 4 ].…”

“…The median age of the diagnosis is ~7 years [ 4 ]. A female predominance with a sex ratio of 3:2 has been described [ 8 ].…”

“…Furthermore, a long-term follow-up including imaging control is necessary to prevent potential relapse. There is no need for neoadjuvant or adjuvant treatment [ 8 ].…”