Primary Extradural Epithelioid Leiomyosarcoma of the Cervical Spine: Case Report and Literature Review

Abstract

ABSTRACTOBJECTIVE AND IMPORTANCE:No case of primary epithelioid leiomyosarcoma involving the spine has been reported previously.CLINICAL PRESENTATION:A 61-year-old Nigerian woman presented with progressive spastic quadriparesis and acute urinary retention. Her only medical history included a total abdominal hysterectomy for fibroids 10 years earlier in Nigeria. Results of the general examination were normal. Pyramidal spastic quadriparesis (3/5) with a sensory level at C5–C6 was found neurologically. Magnetic resonance imaging scans of the brain and spine revealed extradural cord compression at C3–C5 as the sole abnormality. This was caused by a large, soft tissue mass arising from the erector spinae muscles, which was predominantly isointense on T1-weighted images, of mixed intensity on T2-weighted images, and homogenously enhanced after gadolinium contrast agent administration. There was an associated signal change in the cord at C3–C4. Computed tomography confirmed the predominantly soft tissue involvement, but with bone erosion and infiltration within the posterior elements of C4. Systemic screening for cancer was negative.INTERVENTION:At decompressive laminectomy, urgently undertaken under corticosteroid cover, an excessively vascularized, soft tissue tumor was subtotally excised, after which independent walking and normal sphincter function were regained within 1 week. Four weeks later, a complete macroscopic tumor excision was undertaken, incorporating lateral mass (C3–C6) and C2 pedicle screw stabilization, along with iliac crest bone grafting. The patient's neurological status continued to improve. However, while awaiting radical radiotherapy, the patient declined further treatment and returned to her native Nigeria. Histopathological findings were consistent with an epithelioid leiomyosarcoma.CONCLUSION:This is the first reported case of a primary craniospinal epithelioid leiomyosarcoma.