Ganglioneuroma

Case contributed by RMH Report Writing

Presentation

Left sided chest pain in a young lady.

Patient Data

Age: 31 years
Gender: Female
X-ray

Chest radiograph

A large mass is present in the left paravertebral region projected behind the heart on the frontal view. The lungs and pleural spaces are clear. No obvious bony abnormality. The heart size is within normal limits.

CT

CT Chest

There is a left paravertebral mass lesion that extends from the T7 - T11 vertebral levels. The mass is soft tissue density, and also demonstrates small foci of calcification. There is some associated scalloping of the left side of the adjacent thoracic vertebral bodies, indicating a slow growing lesion. No bone erosion or destruction. There is no extension into the neural exit foramen or widening of the intercostal spaces.

The mass is distinct from the oesophagus. lt is closely adjacent to the descending aorta, but the intervening fat plane appears to be preserved.

The lungs are clear.

No anterior mediastinal or hilar adenopathy.

Conclusion: Left paravertebral mass lesion from T7-T11. The appearance favours a neurogenic tumour, likely arising from the sympathetic trunk.

MRI

MRI Thoracic spine

Technique: Multiplanar, multisequence imaging has been obtained through the thoracic spine (non-contrasted study).

Findings: 

There is a paraspinal soft tissue mass, low signal on T1, heterogeneous high signal on T2.

There is an erosion of the adjacent vertebral bodies; there is no extension into the spinal canal.

Conclusion: Paraspinal mass in keeping with a slow growing neurogenic tumour.

PATHOLOGY REPORT

CLINICAL NOTES: Mediastinal mass. Paravertebral.

MACROSCOPIC DESCRIPTION: "Mediastinal mass": Well circumscribed soft grey tissue, 160g, 120x65x45mm. One surface partly lined by shiny membranous pleura over an area 110x70mm. Opposite surface roughened, inked black. Bisected to reveal rubbery soft solid grey tissue with vague nodularity and septa. No areas of necrosis or haemorrhage seen.

MICROSCOPIC DESCRIPTION: Sections show a well demarcated, unencapsulated tumour composed of a proliferation of loosely arranged spindle cells with undulating nuclei and scattered single cells and groups of ganglion cells. The ganglion cells have abundant, amphophilic cytoplasm, large eccentric nuclei and prominent nucleoli. There is a mild patchy lymphocytic infiltrate, scattered adipocytes and occasional psammomatous calcifications. Mitoses are rare. Immature neural elements, haemorrhage and necrosis are not seen. The tumour is 0.4mm from the resection margin but appears completely excised.

DIAGNOSIS: Mediastinal mass: Ganglioneuroma. Completely excised.

Case Discussion

Paraspinal ganglioneuromas are extremely rare benign tumours.

Like neuroblastomas and ganglioneuroblastomas, ganglioneuromas are derived from the primordial neural crest cells that form the sympathetic nervous system.

Ganglioneuromas most commonly arise in the paravertebral sympathetic chains of the posterior mediastinum (41.5%) or retroperitoneum (37.5%). Less common sites include the adrenal gland (21%) and neck (8%). Paravertebral ganglioneuromas frequently extend through the neural foramina to involve the epidural space of the spinal canal.

The differential diagnosis for a posterior mediastinal mass is broad and includes:

  • neurogenic tumours
    • schwannoma
    • neurofibroma
    • malignant peripheral nerve sheath tumour
  • neuroblastic tumours
    • neuroblastoma
    • ganglioneuroma
  • non-neurogenic tumours
    • chordoma 
    • phaeochromocytoma 
  • paraspinal abscess
  • descending aortic aneurysm
  • oesophageal neoplasm
  • hernias 
    • hiatus hernia
    • Bochdalek hernia
  • lymphadenopathy or lymphoma 
  • extramedullary haematopoiesis
  • foregut duplication cysts
    • neurenteric cyst
    • oesophageal duplication cyst
  • thoracic meningocoele
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Case information

rID: 38464
Case created: 21st Jul 2015
Last edited: 22nd Jun 2017
Systems: Chest, Spine
Tag: rmh
Inclusion in quiz mode: Included

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