Showing papers by "Leslie N. Sutton published in 2004"

Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults

Abstract: Purpose The optimal management of craniopharyngiomas remains controversial, especially in children and young adults. This study reports a single institution's experience with such patients. Methods and materials Between 1974 and 2001, 76 patients were treated for craniopharyngioma at the Children's Hospital of Philadelphia and the Hospital of University of Pennsylvania (HUP). Of these, 75 patients (97%) were evaluable with long-term follow-up. Although all patients underwent attempted gross total resection, 27 had documentation of less than total resection with 18 of these patients receiving immediate postoperative radiotherapy (RT). An additional 22 patients received RT at HUP after failing surgery alone. Results Median follow-up for all patients was 7.6 years. The 10-year actuarial overall survival, relapse-free survival, and local control (LC) rates for all patients were 85%, 48%, and 53%, respectively. When comparing the 57 patients treated with surgery alone to the 18 treated with subtotal resection (STR) followed by RT, a significant difference in LC rates at 10 years (42% vs. 84%, respectively; p = 0.004) was noted. However, no statistically significant difference in overall survival was found between the two groups, because RT was highly effective as salvage therapy. Twenty-two patients at HUP treated with RT after relapse had a 10-year ultimate LC rate comparable to that of patients who received RT immediately after STR. Conclusion RT given either immediately after STR or at relapse is effective in controlling craniopharyngiomas.

Craniospinal radiation in the treatment of biopsy-proven intracranial germinomas: twenty-five years' experience in a single center.

Abstract: Purpose The optimal treatment for intracranial germinomas remains controversial. We report on our 25-year experience using craniospinal irradiation (CSI) for this disease. Methods and materials Between September 1976 and May 2001, 39 patients with biopsy-proven intracranial germinomas seen at the Children's Hospital of Philadelphia/Hospital of the University of Pennsylvania received CSI. Thirteen of 36 patients (36%) had evidence of spinal dissemination. Median doses to the whole brain, primary site, and spine were 36 Gy (range, 18–44.2 Gy), 50.4 Gy (range, 44–55.8 Gy), and 30.6 Gy (range, 18–40 Gy), respectively. Results With a median follow-up of 7.1 years (range: 1.5–20.2 years), there have been no documented relapses. This includes 5 patients without spinal dissemination who received 18–19.8 Gy to the craniospinal axis; for these patients, the median length of follow-up was 5.5 years (range, 1.3–6.8 years). One patient, who had no evidence of disease 12.9 years after CSI, died of unknown causes 4 months later. Conclusions Our treatment of intracranial germinomas with CSI has yielded outstanding results with no known relapses during a long follow-up period. These results must be considered when evaluating other approaches, such as chemotherapy only or local field irradiation.

Predictors of progression of scoliosis after decompression of an Arnold Chiari I malformation.

Abstract: STUDY DESIGN Retrospective study of patients with scoliosis and an Arnold Chiari I malformation requiring operative management. OBJECTIVES Determine the factors that could predict whether a particular spinal deformity might progress despite neurosurgical management of Arnold Chiari I malformation. SUMMARY OF BACKGROUND DATA Few studies have documented the relationship between diagnosis and treatment of Arnold Chiari I malformation and associated spinal deformities. Most studies mix neural axis abnormalities and contain limited information about the spinal deformity. METHODS Medical records, radiographs, and magnetic resonance images of patients were evaluated focusing on age and findings at presentation, characteristics of presenting and follow-up spinal deformities, and the specifics of neurosurgical and orthopedic management. Patients were divided into two groups: those whose curves progressed >10 degrees or to surgical range (largest curve >45 degrees ) after neurosurgical intervention (progressors) and those whose curves stabilized or decreased (nonprogressors). RESULTS Eight progressors presented at an average age of 11.4 years (range 2-19) and were followed for 6.3 years (range 2-15). Seven nonprogressors presented at 6.5 years of age (range 5-10) and were followed for 6.6 years (range 3.5-14). Neurosurgical procedures were equivalent in both groups; however, surgical revisions were required in 3 out of 8 progressors and 1 out of 7 nonprogressors. All progressors had a double scoliosis curve; but only one nonprogressor had a double curve. Six out of 8 progressors and 0 out of 7 nonprogressors had a rotation >or=2+ and 50% of progressors had a thoracic kyphosis >50 degrees compared to 1 out of 7 nonprogressors. CONCLUSIONS In this series, progression of spinal deformity after neurosurgical management of Arnold Chiari I malformation was associated with later age at neurosurgical decompressions and initial neurologic symptoms, double scoliosis curve patterns, kyphosis, rotation, and larger curve at presentation.

Treatment of pediatric intracranial arteriovenous malformations with linear-accelerator-based stereotactic radiosurgery: the University of Pennsylvania experience.

Abstract: There are relatively few reports detailing the outcome of children and adolescents with arteriovenous malformations (AVMs) treated with stereotactic radiosurgery (SRS). We reviewed our experience over

Fetal head biometry following in-utero repair of myelomeningocele.

Abstract: Objective To evaluate the impact of prenatal myelomeningocele repair on fetal head biometry. Methods Fifty fetuses underwent open fetal myelomeningocele repair at our institution between January 1998 and July 2002. All had serial head circumference (HC) and lateral ventricular diameter (VD) measurements taken preoperatively and weekly for 8 weeks after repair. Cortical index (CI) was defined as HC/VD. Measurements were compared with gestational age-matched values from nomograms. One-sample t-test, ANOVA and repeated measures analysis were used to assess HC, VD and CI after fetal repair. Results Preoperatively, the HC in fetuses with myelomeningocele was smaller than control values (186.4 vs. 198.8 mm, P = 0.0004). Eight weeks’ postoperatively this difference had resolved (293 vs. 301.6 mm, P = 0.76). The mean increase in CI after repair was 20% (P = 0.02) compared with the predicted 51% in normal cases. The average increase in VD was 3.9 mm (38.8%, P < 0.001). Conclusions Mid-gestational repair of myelomeningocele alters fetal head growth. Increased CI suggests HC changes are not due to ventriculomegaly alone. Copyright  2004 ISUOG. Published by John Wiley & Sons, Ltd.

Intramedullary cervical spine germinoma: Case report

Abstract: Objective and importance We report an extremely rare case of primary intramedullary germinoma in the cervical spinal cord arising in an 18-year-old man who had not undergone previous surgery or irradiation. Clinical presentation The patient had a 2-month history of intermittent neck pain and a 4-week history of bilateral hand paresthesias and weakness. A magnetic resonance imaging scan demonstrated a heterogeneous cervical spine lesion with marked contrast enhancement extending from C3 to C6. Intervention The patient underwent a cervical laminotomy with tumor resection, and pathological examination revealed the tumor to be a germinoma. He recovered well from the surgery with minimal neurological deficits. A postoperative magnetic resonance imaging scan of the brain and spinal cord did not show any other tumors. In addition, imaging studies of the mediastinum, testes, and the rest of the body also did not demonstrate any other tumors. The patient received local radiation as well as three courses of chemotherapy. Conclusion To our knowledge, this is the first report of an intramedullary cervical spine germinoma with confirmed tissue diagnosis. Although extremely uncommon, the possibility of germinoma should be included in the differential diagnosis for primary intramedullary spinal cord tumors.

S-100β Protein – Serum Levels in Children with Brain Neoplasms and its Potential as a Tumor Marker

Abstract: Objective: To determine if serum S-100β levels are elevated in children with brain neoplasms and if it can be used as a tumor marker for children with brain neoplasms. Design: Prospective cohort study. Setting: Urban, tertiary care, children's teaching hospital. Patients: 136 healthy children and 27 children with brain neoplasms. Methods: Serum levels of S-100β were measured in 136 healthy children to serve as controls and 27 children with brain neoplasms, who underwent biopsy or resection of the mass. Patients were then classified into astrocytoma or non-astrocytoma groups. Measurements and main results: The median serum S-100β level for the control group was 0.27 mcg/l (range, 0.06–2.6 mcg/l), and for the brain neoplasm group was 0.2 mcg/l (range, 0.01–2.1 mcg/l), (p = 0.09). There were 13 children with astrocytomas and 14 with non-astrocytomas. The S-100β levels for the astrocytoma group was 0.25 mcg/l (range, 0.05–1.1 mcg/l) and for the non-astrocytoma group 0.17 mcg/l (range, 0.01–2.1 mcg/l), (p = 0.47). Conclusions: Serum S-100β levels are not elevated in children with brain neoplasms compared to healthy children, nor are they elevated in children with astrocytomas compared to non-astrocytomas. The S-100β protein does not appear to be useful as a serum tumor marker in children with brain neoplasms.