Neurofibromatosis type 1 (NF1) is an autosomal dominantly inherited familial tumor syndrome. Benign tumors such as pilocytic astrocytoma, optic glioma make up the majority of intracranial neoplasms in patients with NF1. There have only been a handful of cases in which adult glioblastoma presented with NF1. A 32-year-old male presented with headache and radiological studies showing a high grade intra-axial tumor. The patient underwent gross total surgical excision and the pathology revealed glioblastoma. After the surgery, he received concomitant chemo-radiotherapy with temozolomide and adjuvant temozolomide chemotherapy. We report a NF1 patient who developed glioblastoma and reviewed related articles.
Adult ; Astrocytoma ; Brain Neoplasms ; Drug Therapy ; Glioblastoma* ; Hand ; Headache ; Humans ; Male ; Neurofibromatosis 1* ; Optic Nerve Glioma ; Pathology

OBJECTIVE: This study aims to determine whether gamma knife radiosurgery (GKR) improves survival in patients with recurrent highgrade gliomas.METHODS: Twenty nine patients with recurrent high-grade glioma underwent 38 GKR. The male-to-female ratio was 10 : 19, and the median age was 53.8 years (range, 20–75). GKR was performed in 11 cases of recurrent anaplastic oligodendrogliomas, five anaplastic astrocytomas, and 22 glioblastomas. The median prescription dose was 16 Gy (range, 10–24), and the median target volume was 7.0 mL (range, 1.1–15.7). Of the 29 patients, 13 (44.8%) received concurrent chemotherapy. We retrospectively analyzed the progression-free survival (PFS) and overall survival (OS) after GKR depending on the Eastern Cooperative Oncology Group (ECOG) performance status (PS), pathology, concurrent chemotherapy, radiation dose, and target tumor volume.RESULTS: Starting from when the patients underwent GKR, the median PFS and OS were 5.0 months (range, 1.1–28.1) and 13.0 months (range, 1.1–75.1), respectively. On univariate analysis, the median PFS was significantly long in patients with anaplastic oligodendroglioma, ECOG PS 1, and target tumor volume less than 10 mL (p < 0.05). Meanwhile, on multivariate analysis, patients with ECOG PS 1 and target tumor volume less than 10 mL showed improved PFS (p=0.043 and p=0.007, respectively). The median OS was significantly increased in patients with ECOG PS 1 and tumor volume less than 10 mL on univariate and multivariate analyses (p < 0.05).CONCLUSION: GKR could be an additional treatment option in recurrent high-grade glioma, particularly in patients with good PS and limited tumor volume.
Astrocytoma ; Disease-Free Survival ; Drug Therapy ; Glioblastoma ; Glioma ; Humans ; Multivariate Analysis ; Oligodendroglioma ; Pathology ; Prescriptions ; Radiosurgery ; Recurrence ; Retrospective Studies ; Tumor Burden

OBJECTIVE: To clarify the biological behavior and prognostic factors of brain stem gliomas, the authors reviewed 48 patients with brain stem gliomas treated between 1980 and 1996. PATIENTS AND METHOD: The clinical presentation, tumor location, pathology, and treatment modalities were correlated with the prognosis of these tumors. Male to female ratio was 1.1:1, with the median age of 12.5 years. Thirty patients(63%) were below 15 years old, and 18 patients(38%) were of adult ages. Mean follow up period was 21.8 months. RESULTS: Focal tumors were in 22 cases and diffuse tumors were in 26. All nine midbrain tumors were focal type. Twenty-two(92%) cases among 24 potine tumors were diffuse type and two cases were focal type as dorsally exophytic tumor. Eleven(73%) cases among 15 medullary and cervicomedullary tumors were focal type and four(27%) cases were diffuse type. Pathologically, there were 14 anaplastic astrocytomas, 11 low grade astrocytomas, 4 glioblastomas, 3 oligodendrogliomas, and 1 ganglioglioma. Fifteen tumors were diagnosed only radiologically without histologic verification. Surgery was done in 33 patients(gross total or subtotal removal in 8, biopsy or partial removal in 15, stereotactic biopsy in 10). Radiation therapy was given in 41 patients(conventional in 29, hyperfractionated in 12). Chemotherapy was done in four patients. CONCLUSION: Statistically significant prognostic factors were symptom duration prior to diagnosis(p=0.0004), cranial nerve palsy(p=0.0206), extent of tumor growth(p=0.0219), contrast enhancement(p=0.0226), intratumoral cyst(p=0.047), histopathological grading(p=0.0304), surgery in patients with focal tumors(p=0.0018), and radiation therapy(p=0.0149).
Adolescent ; Adult ; Astrocytoma ; Biopsy ; Brain Stem Neoplasms ; Brain Stem* ; Cranial Nerves ; Drug Therapy ; Female ; Follow-Up Studies ; Ganglioglioma ; Glioblastoma ; Glioma* ; Humans ; Male ; Oligodendroglioma ; Pathology ; Prognosis ; Survival Rate

Apoptosis is a programmed, physiologic mode of cell death that plays an important role in tissue homeostasis. As for the central nervous system, ischemic insults can induce pathophysiologic cascade of apoptosis in neurophils. Impairment of astroctye functions during brain ischemia can critically influence neuron survival by neuronglia interactions. We aimed to elucidate the protective effect of ketamine on apoptosis by energy deprivation in astrocytes. Ischemic insults was induced with iodoacetate/ carbonylcyanide mchlorophenylhydrazone (IAA/CCCP) 1.5 mM/ 20 micrometer or 150 micrometer/2 micrometer for 1 hr in the HTB-15 and CRL-1690 astrocytoma cells. Then these cells were reperfused with normal media or ketamine (0.1 mM) containing media for 1 hr or 24 hr. FITC-annexin-V staining and propidium iodide binding were determined by using flow cytometry. Cell size and granularity were measured by forward and side light scattering properties of flow cytometry system, respectively. An addition of keta-mine during reperfusion increased the proportion of viable cells. Ketamine alleviated cell shrinkage and increased granularity during the early period, and ameliorated cell swelling during the late reperfusion period. Ketamine may have a valuable effect on amelioration of early and late apoptosis in the astrocytoma cells, even though the exact mechanism remains to be verified.
Anesthetics, Dissociative/*pharmacology ; Annexin A5/pharmacology ; Apoptosis ; Astrocytes/metabolism ; Astrocytoma/*drug therapy/pathology ; Brain/pathology ; Carbonyl Cyanide m-Chlorophenyl Hydrazone/pharmacology ; Cell Line, Tumor ; Cell Size ; Cell Survival ; Central Nervous System/drug effects/pathology ; Enzyme Inhibitors/pharmacology ; Flow Cytometry/*methods ; Humans ; Indicators and Reagents/pharmacology ; Iodoacetates/pharmacology ; Ischemia/pathology ; Ketamine/metabolism/*pharmacology ; Light ; Neurons/metabolism/pathology ; Neutrophils/metabolism ; Perfusion ; Propidium/pharmacology ; Scattering, Radiation ; Time Factors ; Uncoupling Agents/pharmacology