Pigmented epithelioid melanocytoma (PEM) of the spine with compression fracture: case report

Abstract

BackgroundPigmented epithelioid melanocytoma (PEM) is a sporadic type of pigmented melanocytic tumor with uncertain malignant potential. PEM arises as a solitary neoplasm that predominantly occurs spontaneously in otherwise healthy patients. Due to its rarity, a gold standard treatment regimen does not exist; however, symptomatic cases should be managed with radiotherapy and surgery.Case presentationA 28-year-old Thai female presented with a sudden onset of back pain and weakness of the lower extremities during the postpartum period. Magnetic resonance imaging demonstrated abnormal soft tissue formation from T4 to T7; it extended to the vertebral bodies, left neural foramina, and posterior columns of T6 and T7. The patient underwent complete tumor debulking, decompressive laminectomy from T4 to T8, and posterior instrumentation from T3 to T10. The histopathology and immunohistochemistry suggested PEM. The patient fully resolved back pain after surgery. Nevertheless, as the patient re-presented with a neurological deficit a few months after the operative intervention, it was decided to perform a surgical resection via an en bloc vertebrectomy. At the one-year follow-up, although the patient reported continued improvement of her back pain, there was no motor power improvement.ConclusionsSpinal cord compression due to PEM is uncommon, especially in adults. Early diagnosis and treatment provide a good prognosis and help to regain lost neurological functions. Complete tumor removal and decompression of the spinal cord must be considered as a treatment strategy. Perioperative radiotherapy and chemotherapy have also been highlighted as treatment modalities for spinal tumors. With our reported case, early operative intervention coupled with radiotherapy produced satisfying outcomes.