A curious cervical spine case: multiple, primary CNS leiomyosarcomas presenting with rapid growth in the immunocompromised patient

Abstract

Introduction: Primary CNS leiomyosarcomas are rare, dural-based intracranial or intravertebral tumors seen in immunocompromised patients and are associated with latent EBV infection. They may mimic a meningioma or schwannoma on imaging but their clinical presentation progresses much more rapidly. Often times, these tumors are hard to distinguish from secondary, metastatic leiomyosarcoma.

Case presentation: A 30-year-old female with congenital HIV presented to clinic with shoulder pain, paresthesias of the right upper extremity and gait instability. She was noted to have a contrast enhancing dural-based spinal canal lesion measuring 1.5 cm at the C1 vertebral level on MRI. Surgery was proposed but patient deferred. She represented to our Emergency Department 1 month later with right-sided hemiparesis and difficulty with ambulation. On repeat MRI, the lesion had grown to 2.6 cm. She was taken to the OR emergently for gross total tumor resection. The histopathology demonstrated a primary CNS leiomyosarcoma. MRI scan of the brain revealed an extra-axial right frontal lobe lesion measuring 1.8 cm which was also treated with subtotal surgical resection followed by proton beam radiotherapy.

Discussion: Primary CNS leiomyosarcomas should be considered in young immunocompromised patients presenting with dural-based spinal cord tumors. Histopathological studies including EBV testing can definitively make the diagnosis. These tumors have an aggressive nature and need to be treated with complete surgical resection to prevent severe neurological deterioration and adjuvant therapy to prevent recurrence.