Ganglioneuroblastoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Vague chest discomfort.

Patient Data

Age: 2 years
Gender: Male
x-ray

In the left posterior mediastinum, there is a well-circumscribed opacity/mass measuring 5.0 x 2.7 cm. There is mild asymmetry of the soft tissues in the left lower neck in comparison to the right.

ct

Centered in the left posterior mediastinum, extending from the inferior aspect of T1 to superior aspect of T5, there is a well-circumscribed, homogeneous soft tissue mass without focal areas to suggest necrosis or hemorrhage. There are two questionable punctate hyperdense foci, which may represent calcifications. The mass abuts the proximal descending aorta inferomedially and the left subclavian artery superiorly. There is no mass effect, stenosis, or encasement of these vessels. The mass is closely approximated to the neural foramen from T2-T5, however there is no definite evidence of deeper invasion into the spinal canal. There is no mass effect on the spinal cord. The adjacent vertebral bodies and left ribs are normal in attenuation without areas of cortical disruption or periosteal reaction.

Case Discussion

This is a case of pathologically-proven ganglioneuroma. Pathological analysis demonstrates a schwannian stroma-predominant neuroblastic proliferation, composed of randomly-distributed microscopic foci of neuroblastic cells embedded in neuropils. The neuroblastic cells were predominantly differentiating neuroblasts including ganglion-like cells and, rarely, less differentiated neuroblasts. The mitosis-karyorrhexis index was low and no distinct nodules were identified. In immunohistochemical stains, S100 and SOX10 highlights schwannian stroma, a subset of neuroblasts are positive for neurofilament and chromogranin, and the neuropil is highlighted by synaptophysin. The findings are consistent with ganglioneuroblastoma, intermixed subtype.

The patient had an uneventful postoperative course and was discharged home on post-op day 1. Patient has been followed as an outpatient, noted to have a persistent left Horner’s syndrome.

Co-authors:
Alannah McCann
Travis Bevington, MD

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.