Retrospective Analysis of 28 Cases Confirmed for Primary Angiitis of the Central Nervous System by Biopsy.

OBJECTIVE: The present study aimed to summarize the clinical characteristics, therapeutic effects, and long-term prognosis of cases confirmed with primary angiitis of the central nervous system (PACNS) by biopsy, analyze the risk factors, and provide clinical guidance for the diagnosis and treatment of the disease. METHODS: Retrospective analysis was performed on 28 cases of PACNS confirmed by biopsy, and the age, gender, pathological results, course of the disease, imaging manifestations, treatment, and prognosis of the patients were analyzed and summarized. RESULTS: The cohort (age 16-60 years) comprised of 16 males. The average time from the visit to diagnosis was 6 months. The first symptom was chronic headache in 18 patients. The pathological results were accompanied by demyelination in 10 cases and glial hyperplasia in 6 cases. A total of 27 patients received treatments including glucocorticoid+cyclophosphamide; of these, 3 cases of craniotomy were improved. Among the 28 patients, 15 patients improved after the treatment, 12 patients had no significant improvement, and 1 patient was deceased. Patients with a long course of the disease before diagnosis, a Karnofsky performance status (KPS) score <60 at the time of diagnosis, a behavioral, cognitive abnormality before treatment, and a short-term relapse (0.3-1 month) have a poor outcome. CONCLUSIONS: PACNS patients are prone to misdiagnosis and mistreatment, with unknown etiology and poor prognosis due to delayed treatment. Therefore, early biopsy, pathological diagnosis, and timely treatment with glucocorticoid shock are recommended, and patients with obvious mass effect should be treated by surgical resection.

[Mixed craniopharyngioma: long-term results after gamma knife combined with stereotactic brachytherapy].

OBJECTIVE: To evaluate the long-term results of combination treatment with Gamma Knife radiosurgery and stereotactic intracavitary brachytherapy for mixed cystic and solid craniopharyngiomas. METHODS: Sixty-seven consecutive patients with mixed cystic and solid craniopharyngioma treated by Gamma Knife radiosurgery combined with stereotactic brachytherapy from October 1996 to December 2005 were selected for retrospective analysis. The inclusion criterion was the patients who survived for at least 5 years after combined treatment. There were 39 male and 28 female patients and the mean age was 31.5 years (ranged from 3 to 70 years). The clinical evaluations including neurological, neuro-ophthalmological, and neuro-endocrinological examinations, assessment of comprehensive quality of life and neuroimaging examinations were performed periodically. The actuarial survival rates and the mean survival time were calculated by using Kaplan-Meier product limit method. The rates were compared using the χ(2) test. RESULTS: Follow-up period varied from 60 to 168 months, with an average of 114 months. The tumor response rate gained from combination treatment with Gamma Knife radiosurgery and stereotactic intracavitary brachytherapy for predominantly solid and cystic craniopharyngiomas were 10/12 and 90.9% respectively, and 89.6% in all. Mean survival after combination treatment was (110 ± 9) months. The mean survival of patients with predominantly solid and cystic craniopharyngioma were (97 ± 12) months and (120 ± 14) months and the actuarial 10-year survival rates were 7/12 and 69.1%. There was no statistics difference in tumor response rate and 10-year survival rate between 2 groups of patients with predominantly solid and cystic craniopharyngioma. The actuarial 5-, 6-, 7-, 8-, 9- and 10-year survival rates were 90.5%, 85.7%, 83.3%, 76.4%, 69.4% and 60.0% respectively. The decreased visual acuity had improved in 68.3% at 6 months postoperatively and in 70.0% in long term results. Comprehensive quality of life in long term follow-up of 67 patients was excellent in 28 cases(41.8%), good in 19 cases(28.4%), fair in 17 cases(25.4%) and poor in 3 cases(4.5%), respectively. The side effects that occurred 6 to 12 months after treatment were worsening of visual acuity (4 patients), dysfunction of hypothalam (4 patients) and third nerve palsy was found in 1 patents 5 years after treatment. The rate of complications was 13.4%. CONCLUSION: Combination treatment with Gamma Knife radiosurgery and stereotactic intracavitary brachytherapy is highly effective and safety in the treatment of mixed cystic and solid craniopharyngiomas.