Abstract:Paravertebral ganglioneuroma and scoliosis is a rare clinical benign disease. The case we reported is about a 12-year-old girl who was hospitalized due to neoplasm with spinal deformity in the right abdomen for 1 month. Based on a careful preoperative evaluation and found no obvious surgery contraindications, the patient was treated with surgical resection of the tumor and correction of the deformity by surgery. Postoperative pathologic examination confirmed it was a ganglioneuroma. After the operation, the pa… Show more
“…The management of the co-presenting scoliosis is variable. Yang et al advocated for separation of excision and spinal fusion into two stages with a one- to seven-week interval in between to enable a shorter operation time [ 5 ]. Of the 18 cases reviewed, five patients were treated with this approach.…”
Ganglioneuromas are rare, benign tumors arising from the sympathetic nervous system. The presentation of the tumor is variable and may be associated with scoliosis. Few reports of ganglioneuroma associated with scoliosis exist and most involve the thoracic spine. Here, we present a 13-year-old female with scoliosis who was found to have a lumbar ganglioneuroma. The patient was treated with a subtotal resection and lumbar spinal fusion to correct her scoliosis in a single-stage operation. The patient's symptoms and scoliosis markedly improved following treatment without any complications. Additionally, we conducted an up-to-date literature review of ganglioneuromas associated with scoliosis that have been published in the last 20 years. We discuss variations in clinical presentation and surgical approach.
“…The management of the co-presenting scoliosis is variable. Yang et al advocated for separation of excision and spinal fusion into two stages with a one- to seven-week interval in between to enable a shorter operation time [ 5 ]. Of the 18 cases reviewed, five patients were treated with this approach.…”
Ganglioneuromas are rare, benign tumors arising from the sympathetic nervous system. The presentation of the tumor is variable and may be associated with scoliosis. Few reports of ganglioneuroma associated with scoliosis exist and most involve the thoracic spine. Here, we present a 13-year-old female with scoliosis who was found to have a lumbar ganglioneuroma. The patient was treated with a subtotal resection and lumbar spinal fusion to correct her scoliosis in a single-stage operation. The patient's symptoms and scoliosis markedly improved following treatment without any complications. Additionally, we conducted an up-to-date literature review of ganglioneuromas associated with scoliosis that have been published in the last 20 years. We discuss variations in clinical presentation and surgical approach.
“…A Japanese study indicated a good prognosis postoperatively [6]. GN is mostly found in women, with a female:male ratio of 3:2 [8]. The preoperative diagnosis of GN is often difficult [6].…”
Ganglioneuroblastoma, neuroblastoma, and ganglioneuroma (GN) are the tumors that arise from the neural crest cells. Of these, GN has the most benign origin without metastatic potential. The most common sites of their origin are the posterior mediastinum and retroperitoneum. Although the imaging studies, including CT, are available to detect these tumors, the definitive diagnosis can only be made by histological examination. We present a case of a 40-year-old woman with a retroperitoneal GN causing longstanding, gradually increasing, uncontrolled abdominal pain due to its pressure effect on the pancreas, duodenum, and right kidney with the displacement of the inferior vena cava. An exploratory laparotomy was performed, and the mass was removed. Histopathology confirmed the benign nature of the mass (a GN). These tumors are rarely malignant and mostly asymptomatic. However, in our case, abdominal pain was affecting the patient's life. After a discussion with the patient, an elective surgical procedure was performed, and the patient was symptom-free postoperatively and able to resume her regular routine.
“…TR None No (12) 2013 [ 31 ] 12 ♀ Presacral 12 Open TR None No (12) 2017 [ 32 ] 12 ♀ Thoracic 12 Open PR (2) None No (12) 2014 [ 33 ] 66 ♀ Thoracic 12 n.a. Pain, hypertension No (36) 2016 [ 5 ] 12 ♀ Retroperitoneal 13 Open PR (8) None n.a. FU Follow-up in months, TR Total resection, PR Partial resection, DM Maximum diameter (in cm) a Citations in brackets b Article in Japanese c Completion in weeks Fig.…”
Section: Methodsmentioning
confidence: 99%
“…Total resection, on the other hand, was performed in 10 out of 13 cases. In the remaining three cases, one was removed piecemeal in one procedure, whereas a second surgical approach was necessary in the remaining two cases [5,32]. Twelve out of 13 papers reported the absence of surgical complications or postoperative unexpected events.…”
Section: Systematic Literature Reviewmentioning
confidence: 99%
“…The median age at the time of the diagnosis is reported to be approximately 7 years [ 4 ]. Ganglioneuromas, in general, occur more frequently in females than in males with a ratio of about 3:2 [ 5 ]. As these tumors are generally diagnosed due to compressive symptoms and consequently resected in children, there is usually a natural limit to tumor size given by the available space within the body cavity.…”
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.
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