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Ganglioneuroma

Case contributed by Melbourne Uni Radiology Masters
Diagnosis certain

Presentation

Left sided chest pain in a young lady.

Patient Data

Age: 30 years
Gender: Female

Chest

x-ray

There is a large mass 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.

Chest

ct

The left paravertebral mass lesion that extends from the T7 - T11 vertebral levels, has 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 esophagus. lt is closely adjacent to the descending aorta, but the intervening fat plane appears to be preserved.

The lungs and pleural spaces are clear.

No anterior mediastinal or hilar adenopathy.

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

Thoracic spine

mri

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 chronic scalloping of the adjacent vertebral bodies but no erosion; there is no extension into the spinal canal.

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

PATHOLOGY REPORT

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 hemorrhage seen.

MICROSCOPIC DESCRIPTION: Sections show a well-demarcated, unencapsulated tumor 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 are a mild patchy lymphocytic infiltrate, scattered adipocytes and occasional psammomatous calcifications. Mitoses are rare. Immature neural elements, hemorrhage and necrosis are not seen. The tumor 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 tumors.

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 tumors
    • schwannoma
    • neurofibroma
    • malignant peripheral nerve sheath tumor
  • neuroblastic tumors
    • neuroblastoma
    • ganglioneuroma
  • non-neurogenic tumors
    • chordoma 
    • pheochromocytoma 
  • paraspinal abscess
  • descending aortic aneurysm
  • esophageal neoplasm
  • hernias 
    • hiatus hernia
    • Bochdalek hernia
  • lymphadenopathy or lymphoma 
  • extramedullary hematopoiesis
  • foregut duplication cysts
    • neurenteric cyst
    • esophageal duplication cyst
  • thoracic meningocoele

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