Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report.

OBJECTIVE AND IMPORTANCE: Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis. CLINICAL PRESENTATION: We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance. INTERVENTION: Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG. CONCLUSION: Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.

Leiomyosarcomatous transformation of unknown pediatric primary brainstem lesion following radiotherapy. Case report.

Primary leiomyosarcoma of the central nervous system is rare and has been described both de novo and following temporally remote radiotherapy for a different unrelated malignancy. The authors report the case of a 42-year-old man in whom 60Co radiation treatment had been performed for an unknown primary mass in the brainstem 25 years previously. He presented with progressive neurological deterioration after undergoing many years of conservative therapy. A stereotactic biopsy sampling procedure was performed, and examination of the left cerebral pedunculopontine lesion revealed a spindle cell neoplasm. Histopathological and immunohistochemical examination of the tumor obtained from definitive resection suggested leiomyosarcomatous transformation of ganglioglioma.

Treatment of atlantoaxial instability in pediatric patients.

The atlantoaxial region has been extensively described as a spinal segment especially prone to injury in children. In this clinical review, the authors evaluate and summarize the management of 23 pediatric cases of atlantoaxial instability treated between March 1990 and October 2002. Four broad categories of atlantoaxial problems were observed-atlantoaxial rotatory subluxation in six patients, anterior-posterior atlantoaxial instability caused by ligamentous injury or congenital ligamentous laxity (10 patients), atlantoaxial fracture with or without dislocation (five patients), and atlantooccipital dislocation (two patients). Most cases (60.9%) were treated without surgical intervention, resulting in excellent outcomes; however, 21.7% of cases were treated with a cervical halo (mean patient age 72.6 months) alone for 3 months. Various techniques of surgical stabilization including transarticular screws with sublaminar wiring, transoral decompression with posterior plating, and laminectomy with Steinmann pin occipital-cervical fusion were used with good results. Both patients with atlantooccipital dislocation underwent immediate Locksley occipital-cervical fusion, with marked neurological improvement. Individualized case management must be based on clinical presentation, with internal fixation being the last resort.