Gross total resection is suggested as the optimal management

Gross total resection is suggested as the optimal management for intracranial gangliogliomas. The 10-year survival rate for patients with supratentorial ganglions can be as high as 85%. However, total resection of brainstem gangliogliomas is risky and some important neuronal structures are involved in the lesion. Some physicians may consider adjuvant or salvage radiotherapies. Because of their rarity, the benefits of postoperative radiotherapy remain controversial. No prospective studies have been performed regarding radiotherapy for gangliogliomas—even for supratentorial gangliogliomas. In 2012, the Mayo Clinic group reported a large series of 88 ganglioglioma patients who had been treated since 1970; only a small group of patients (n = 7) underwent radiotherapy postoperatively. The results proved that adjuvant/salvage radiation therapy was not associated with prolonged progression-free survival for the group as a whole (p = 0.74). Most other reports agree that gross total resection should be performed whenever safely possible and that this does not require postoperative irradiation. Even partial resections of brainstem gangliogliomas also have a favorable long-term outcome with a 5-year survival rate of 78%.
Introduction Fibrous dysplasia (FD) is usually a benign fibro-osseous developmental anomaly caused by the replacement of the medullary component of one or several bones with fibrous dna ligase and irregular osteoid formation (malignant transformation occurs in <1% of cases). This disease primarily affects adolescents and young adults and accounts for 7% of benign bone tumors. Most lesions occur in the ribs or craniofacial bones, especially the maxilla. Fibrous dysplasia commonly presents in a monostotic form or may present in a polyostotic form (25% of people with the polyostotic form have >50% of the skeleton involved with associated fractures and skeletal deformities). Fibrous dysplasia is often subcategorized based on the number of bones involved. Monostotic lesions involve only one bone, whereas polyostotic lesions involve many bones. Many asymptomatic lesions are discovered incidentally; the remainder present with the symptoms of swelling, deformity, or pain. The polyostotic form of FD has been associated with McCune–Albright syndrome and Mazabraud\'s syndrome, in which skeletal abnormalities are associated with characteristic café au lait spots and endocrinal abnormalities. The etiology remains unclear. Fibrous dysplasia has a characteristic radiographic appearance. Most patients do not require intervention, but some patients are managed surgically with curettage, bone grafting, and (in some patients) internal fixation. We report a rare case of FD of the thoracic spine and review the literature.
Case Report Plain radiographs of the cervical and thoracic spine demonstrated an osteolytic lesion of the left T1 and the left first rib with a pathologic compression fracture of these bones, which suggested a metastatic tumor (Fig. 1). Computed tomography (CT) of the thoracic spine showed osteolytic lesions that involved the vertebral body with ground-glass opacity, the laminae on both sides, the spinous process of T1, and the left first rib (Fig. 2). Magnetic resonance imaging (MRI) demonstrated a multiloculated lesion with septa-like structures and marginal sclerosis extending from the T1 vertebral body to the adjacent left posterior part of the first rib. This lesion showed low signal intensity on T1-weighted and T2-weighted images with mild enhancement. We therefore highly suspected FD. A herniated C5–6 intervertebral disc with posterior osteophyte caused mild thecal sac compression and bilateral neural foraminal encroachment (Fig. 3). The differential diagnosis of the lytic bone lesions included primary neoplasm, secondary neoplasm, or an infectious process. We suggested an en bloc resection of the tumor for decompression and pathological diagnosis. During the operation, the bone tumor was white, elastic, and did not exhibit the expected hypervascularity. Surgical treatment consisted of C5–6 diskectomy with artificial cage fusion [Bryan® (Medtronic Spinal and Biologics, Memphis, Tennessee)], en bloc tumor resection, T1 corpectomy with C7–T2 mesh and plate fixation. Pathological examination of the surgical specimen revealed curvilinear trabeculae of woven bone arising in the background of fibrous tissue (Fig. 4). There was no evidence of malignancy in the specimen. We also performed bone scintigraphy, which did not show disease at any other site.