A giant ganglioneuroma accompanied with scoliosis

Abstract

Ganglioneuroma is a rare benign neoplasm. Patients with ganglioneuroma show no symptoms. We describe a rare case of giant ganglioneuroma with scoliosis in a 35-year-old woman, who presented to our hospital for haemoptysis. We combined with neurosurgeons to remove the tumour successfully. After 16 months of follow-up, there is no evidence of tumour recurrence.

Background

Ganglioneuroma is a rare benign neurogenic neoplasm incorporate with ganglion cells and mature Schwann cells and nerve fibres. Ganglioneuromas are usually found in the posterior mediastinum or retroperitoneum of patients without any symptoms.¹ Although it is a benign tumour, it can grow stupendously large and can cause severe complications. We report a case of a 35-year-old woman with secondary scoliosis who presented with recurrent haemoptysis due to a large ganglioneuroma in the left mediastinal cavity.

Case history

The patient was referred to the thoracic surgery department of our hospital due to recurrent haemoptysis. Computed tomography (CT) of the thorax performed elsewhere revealed a huge mass pressing against the spine in the left thorax and scoliosis. An enhanced CT (Fig 1A,B) and nuclear magnetic resonance imaging (MRI) of the thorax were performed in this hospital and showed a 16cm well-circumscribed tumour spreading into the spinal canal through the intervertebral foramina in the left middle and lower thorax, and scoliosis with the enlargement of the intervertebral foramina of T4–T7 (Fig 1C,D) This patient did not complain any other neurological symptoms and had no family history of similar disease.

Figure 1.

Computed tomography (CT) and magnetic resonance imaging (MRI) imaging: (A) coronal CT; (B) axial enhanced CT; (C) coronal MRI; (D) axial MRI imaging demonstrated that there was a mid-posterior mediastinum mass located in the left chest and invading the thoracic vertebra.

Pathological examination of a biopsy specimen obtained by CT-guided puncture disclosed a ganglioneuroma. Pulmonary function tests and routine blood examinations for surgery, as well as the electrocardiogram, showed no contraindications to surgery. We held a consultation with neuro- and orthopaedic surgeons to ensure a complete resection of the tumour and redress management of the scoliosis. Considering both the general condition and the age of the patient, the orthopaedic surgeon did not recommend surgical management for the scoliosis.

The patient was placed in the prone position and the neurosurgeons began the operation through an incision at T4–T7. The tumour did not invade the dura and was resected completely from the thoracic vertebrae. The patient was then placed in the right lateral position and thoracic surgeons continued the operation through an incision at the fifth intercostal space. The tumour was enormous and difficult to separate, which resulted in severe bleeding at the surgical wound. Nevertheless, the tumour (approximately 16 × 10 × 6cm) was completely removed from her thorax. The operation last about 150 minutes and blood loss was estimated to be 1200ml.

The specimen was sent to the pathology department for further examination. The immunohistochemistry report described as: S100(+), SOX–10(+), PGP9.5(+), Neu–N(–), NSE(+), Ki–67(–). The postoperative period was uneventful and the patient was discharged 14 days after the operation. There is no evidence of tumour recurrence after 16 months of follow-up (Fig 2).

Figure 2.

(A) Haematoxylin–eosin staining (magnification × 200) revealed that there were mature Schwann cells, ganglion cells and nerve fibres. (B) S100. (C) SOX-10. (D) PGP9.5 immunohistochemistry (magnification × 200) illustrate that S100-stain, SOX-10-stain and PGP9.5 stain were all positive, which confirmed the diagnosis of ganglioneuroma.

Discussion

Owing to the benign nature of ganglioneuroma, it usually does not cause any symptoms. It is not until the tumour grows large enough to compress the adjacent tissues that it causes some pulmonary or cardiac symptoms. In addition, although ganglioneuroma is a benign neurogenic tumour it can become malignant. There are cases where ganglioneuroma has transformed into neuroblastoma and other aggressive soft-tissue neoplasms.² Wang reported a series of patients with ganglioneuroma accompanied by scoliosis.³ Most of these patients were treated surgically.³ As surgical removal is usually a safe and curative treatment, ganglioneuromas should be removed as soon as they are detected. With the development of thoracic surgery, the majority of posterior mediastinal tumours can be removed by thoracoscopic surgery.⁴ In this case, however, the tumour was too big to remove using a thoracoscopic approach, because this patient had not had a medical examination for years, possibly due to the restriction of her economic circumstances and health consciousness. Imaging examinations such as CT and MRI can easily reveal the tumour and describe the characteristics of ganglioneuroma, but a confirmed diagnosis of ganglioneuroma still relies on pathological biopsy and immunohistochemistry. We have followed this patient for 16 months without obvious manifestations of recurrence and metastasis (Fig 3). Further follow-ups still require her to have a chest CT annually.

Figure 3.

Computed tomography 16 months post-surgery, showing that the tumour has been completely removed with no recurrence.

Conclusion

In conclusion, this case of giant ganglioneuroma accompanied by scoliosis is extremely rare. Careful preoperative preparation and skilled surgical skills can make surgery safe for patients. Surgery can be a safe and curative treatment for patients with ganglioneuroma.