Giant Thoracoabdominal Ganglioneuromma : an unusual mimicker of ruptured Aortic Aneurysm in Emergent Setting

1. Giant Thoracoabdominal Ganglioneuromma : an unusual
mimicker of ruptured Aortic Aneurysm in Emergent Setting
V Arora1, N N Pandey1, S Kumar1 , A S Malhi1
Department of Cardiovascular Radiology and Endovascular Interventions, AIIMS , New Delhi

2. INTRODUCTION
Ganglioneuromma (GN)
 rare benign tumor that originates from neural crest cells of sympathetic ganglia
or adrenal medulla
 usually occur in the retroperitoneum (especially presacral space), posterior
mediastinum, or adrenal gland, and occasionally in unusual locations.
Although GNs are commonly benign masses, they can grow aggressively and present
with symptoms of compression of adjacent structures
We present a case of fifty five-year-old adult male with giant thoracic
ganglioneuromma causing encasement of aorta with marked mass effect and
displacement of mediastinal structures characterised using both CT and MRI.

3. CASE DESCRIPTION
A fifty five-year-old adult male presented with complaints of sudden
onset chest pain radiating to back since past three months with recent
worsening. He also had history of single episode of syncope and
dysphagia to solid foods.
TTE was done which revealed extrinsic compression of bilateral atria,
normal ejection fraction with mild AR.
A plain Chest Xray showed a large homogenous opacity extending to
both sides of mediastinum with sharp lateral margins. Superiorly, the
radio-opacity was seen reaching upto D3 /D4 vertebral levels with
obscured inferior margin showing positive thoraco-abdominal sign.

4. RADIOLOGIC FINDINGS
 CTA revealed a homogenous minimally enhancing
soft tissue density posterior mediastinal mass
extending into bilateral para-spinal region.
 Few specks of early calcification (yellow arrow)
were also noted within the mass.
 The mass showed an oblong cranio-caudal
orientation with encasement of thoracic and
supraceliac aorta.
 No fat density/ septae seen within the mass lesion.
 Anteriorly the mass showed abutment with
mediastinal structures without any obvious
infiltration.
 Marked mass effect with anterior displacement of
trachea, oesophagus, cardia and great mediastinal
vessels was noted.

5. RADIOLOGIC FINDINGS
MRI revealed an encapsulated paraspinal mass
showing whorled appearance with heterogeneously
hyperintense signal on T2/STIR images and a T1/T2
hypointense capsule.
Patchy areas of T1 hyperintensity showing no
suppression on fat saturated sequences were
observed.
Post contrast T1WI show patchy areas of minimal contrast
enhancement (asterisk) with mild progression on delayed scans.
No intra-spinal extension of the mass was noted.
Biopsy could not be performed due to sudden cardiac arrest and
death of the patient. In view of the age and clinical
presentation, above imaging findings are indicative of a Giant
Thoraco-abdominal Ganglioneuromma.

6. DISCUSSION CONCLUSION
REFERENCES
 Reeder LB. Neurogenic tumors of the mediastinum, Semin Thorac Cardiovasc Surg, 2000, vol. 12 (pg. 261-267)
 Lambdin JT, Lee KB, Trachiotis G, Picone C (2018) Massive thoracic ganglioneuroma with significant mass effect
on left hemithorax. Case Reports 2018
 Giant thoracic ganglioneuromas are rare.
However, in our case complete
encasement of aorta by ganglioneuroma
is seen which has not been previously
reported in literature.
 This case report highlights the
importance of orthogonal imaging in
characterising mediastinal
ganglioneuromma with characteristic
findings on CT and MRI that help to
distinguish it from other pathologies.
The ganglioneuroma is a rare benign nerve tumor of neuro-
ectodermal origin.
It is estimated to characterize 0.1 to 0.5 % of total central nervous
system (CNS) tumors.
On review of current literature, this is the second largest
ganglioneuroma reported in an adult patient measuring 22 x 11 x
8 cm (CCx TRVx AP extent). So far, the largest tumor has been
reported in an adult patient (24 × 10 × 10 cm) by Lambdin, et al.
The thoracic ganglioneuroma represents a diagnostic challenge
and creates a problem of differential diagnosis with other intra-
thoracic tumors. Notably neurogenic tumors including other
neuroblastic tumors (ganglioneuroblastoma and neuroblastoma)
and nerve sheath tumors (mainly neurofibroma).
Complete surgical removal is recommended for symptom control
or for the prevention of potential malignant degeneration.