Abstract. The majority of giant cell tumors (GCTs) occur in the ends of the long bones. The presence of more than one GCT in the axial skeleton is rare. A GCT is capable of remaining clinically latent following treatment and becoming active a number of years later. We report an extremely rare case of GCT occurring in the axial skeleton, involving the sacrum, thoracic spine and parieto-occipital skull in more than 15 years of follow-up.
IntroductionGiant cell tumors (GCTs) are benign tumors commonly occurring at the ends of the long bones, representing approximately 5% of bone tumors (1). GCTs rarely occur in the spine, with 2-5% of tumors found in the spine above the sacrum (2,3). When occurring in the spine, these tumors most commonly present with localized pain and swelling of the affected side, and may also result in neurological deficit (3,4).In this study, the authors present an extremely rare case of GCT occurring in the axial skeleton, involving the sacrum, thoracic spine and parieto-occipital skull in more than 15 years of follow up. The study was approved by the Institutional Review Board (IRB) for Human Subjects Research and Ethics Committees of Hanyang University Guri Hospital, Korea.
Case reportIn March 1993, a 24-year-old male without a significant past medical history presented with a several-month history of localized pain in the right buttock area. There was induration on the buttock and a firm, palpable mass.Neurological examination revealed mild weakness of the flexor hallucis longus and flexor digitorum longus with grade 4/5 power. The patient experienced diminished sensation in the perineal region, and sphincter tone was slightly diminished. Radiographs revealed a large, osteolytic lesion involving almost the entire sacrum (Fig. 1). Computed tomography (CT) also revealed a destructive sacral mass involving almost the entire sacrum below the S1 vertebral body. T2-weighted magnetic resonance imaging (MRI) showed an expansile soft tissue mass with a heterogeneous signal density. The tumor infiltrated into the spinal and sacral canal, and into the presacral area above S2 (Fig. 2). The technetium-99m methylene diphosphonate whole-body bone scan revealed an abnormally high uptake in the sacral region (Fig. 3). Metastases were not found. To prevent an improper diagnosis or a delay in treatment occurring, open biopsy was performed from the posterior aspect of the sacrum, and histopathological examination revealed numerous multinucleated giant cells within a background of scant stroma consistent with GCT (Fig. 4).With the goal of pain relief, tumor resection and prevention of further neurological deterioration, the patient underwent high sacral amputation using a staged anterior and posterior approach. In the first stage, the anterior procedure with a transperitoneal approach was performed. The anterior aspect of the tumor was exposed, and internal iliac and middle sacral vessels were ligated. The anterior osteotomy was performed through the lower border of the S1 vertebral body at the level just belo...