Intraosseous Melanotic Schwannoma in the Sacrum Mimicking Primary Bone Tumor

Primary tumors of sacrum are rarely seen, and the differential diagnosis is extensive, such as chordomas, giant cell tumors, and schwannomas. Sacral intraosseous schwannomas (IOSs) are very rare and encompass approximately 1%–5% of all spinal schwannomas. Melanotic schwannomas (MSs) are categorized as an unusual variant of benign schwannomas; however, they sometimes follow a malignant course. The authors present a case of MS with intraosseous extension into sacrum in a 48-year-old male arising from the left S2 nerve root. Magnetic resonance imaging (MRI) and computed tomography (CT) scan demonstrated a destructive mass in the sacrum. He was made a diagnosis with MS by 18F-fluoro-deoxy-glucose positron-emission-tomography ((18)F-FDG PET) and open biopsy. The tumor was blackish-colored and vascular-rich fragile tumor covered by fibrous capsule. The floor of the tumor was not encapsulated and invading into the sacral bone. Total removal of the tumor together with the left S2 nerve of origin via posterior approach was achieved. The patient made dramatic recovery of neurological symptoms and tumor recurrence is not seen for 6-month follow-up period. MS is a benign tumor with potential for aggressive behavior and capacity to metastasize. Therefore, total removal of the tumor and careful postoperative follow-up are recommended. Postoperative spinopelvic stability also needs to be taken into consideration. The authors discuss our successful management with a focus on diagnostic process, surgical planning, and histological consideration to provide the most up-to-date guidance on managing this challenging tumor.