Multiple schwannomas

Case contributed by Bruno Di Muzio
Diagnosis almost certain

Presentation

Back pain. No septic or inflammatory markers.

Patient Data

Age: 19 years
Gender: Male

Chest Radiograph

x-ray

The lungs and pleural spaces are clear. There is a mass-like rounded opacity projected within the right paratracheal stripe and another similar one bulging the right lateral spinal contour over the heart silhouette. These findings are non-specific and suspicious for posterior mediastinal/paraspinal masses; correlation with previous imaging is recommended in fist instance, if not available further CT Chest recommended. A lateral projection can be useful to confirm the location, but it will not clarify the etiology.

Differentials, in this case, would include:

  • neurogenic tumors
    • nerve sheath tumors (e.g. schwannoma and neurofibroma)
    • parasymphathetic ganglion tumors
    • sympathetic chain tumors (e.g. neuroblastoma, ganglioneuroblastoma, ganglioneuroma)
  • lymphoma
  • metastasis
  • extramedullary hematopoiesis

CT Chest, Abdomen, and Pelvis

ct

There are multiple soft tissue masses associated with the paraspinal region of the thoracic spine, some extending into the neural exit foramen consistent with nerve sheath tumors. The largest is at the level of the right T3/T4 level extending into the paravertebral region and measures 3.3 x 3.4 cm. There is an expansion of the foramen. Further lesions are seen in the left T1/T2 and left T4/T5 levels. Soft tissue nodule associated with the left 6th intercostal nerve. There is a soft tissue mass within the posterior mediastinum at the level of the T9 vertebral body. This mass measures 2.2 x 3.5 cm in orthogonal planes. There is a mass associated with the right of 10th intercostal space posterolaterally measuring 4.8 x 2.4cm in orthogonal planes. Bilateral lesions at L4 and L5 which extend into the neural exit foramen. Probable subcutaneous lesion seen within the right axilla (partially imaged), right posterior chest wall (paraspinal) and postero lateral right chest wall. There is a nodule measuring 8 mm superficial to the right external oblique muscle. Low density lesion measuring 1.3 cm within the midpole of the right kidney is indeterminate. The remaining solid organs of the upper abdomen are unremarkable. No free fluid identified within the peritoneal cavity.

Case Discussion

This case demonstrates multiple nerve sheath tumors through the chest, abdomen, and pelvis, predominantly within the spinal neural foramina. They are consistent with schwannomas, as it was later revealed a previously known history of neurofibromatosis type II; the patient has also bilateral acoustic schwannoma, one partially resected and histologically confirmed, and multiple intracranial meningiomas. 

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