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.

Keywords: Case report; Ganglioneuroma; Largest; Systematic review; Thoracoabdominal.

Fig. 1

Pedigree with aspect to malignant diseases. Relatives who developed tumor diseases are marked black. Circle, female; square, male

Fig. 2

CT images and 3D reconstruction. The GN (green) extends from the retroperitoneal space through the aortic hiatus into the posterior mediastinum and surrounds (a, c–e) and displaces (b) Inferior vena cava , aorta , tumor

Fig. 3

Macroscopic and microscopic findings. a Macroscopic specimen of the in toto resected GN. b, c H&E-stained histopathological images of the central tumor portion with ganglion cells and stromal tissue as well as myxofibrotic and fat tissue in the transition zone. H&E, hematoxylin and eosin staining

Fig. 4

PRISMA consort diagram of how the literature was screened. *Trivial cases of gastric or esophageal cancers as well as spinal cord surgery, which are naturally located thoracoabdominal (e.g., T1–L2 in spinal dumbbell tumors), were excluded. **Mostly thoracoabdominal approach for solely intraabdominally located tumors