| Objective: We analysis the clinical characteristics of gliomatosis cerebri, and investigate the diagnosis and treatment methods of gliomatosis cerebri, so to improve the diagnosis and treatment levels of gliomatosis cerebri.Method: We selected patients with gliomatosis cerebri confirmed by stereotactic biopsy by screening the data of 1039 patients diagnosed by stereotactic biopsy in The Navy General Hospital of PLA from 1988 to 2007 and 10 patients diagnosed by stereotactic biopsy in Chinese-Japan Union Hospital of Jilin University from January 2005 to December 2007. The diagnosis of GC was based on the following criteria: MRI evidence of a diffuse infiltrative process involving more than two cerebral lobes and often extending to infratentorial structures; Biopsy confirmation of astrocytic or oligodendrocytic proliferation according to the WHO classification of tumors. We excluded diffuse infiltration occurring in the evolution of focal gliomas and recurrence of focal gliomas after surgical resection. 17 cases have been choosed. And clinical data of the 17 cases were analyzed retrospectively. Of the 17 cases, 10 were male and 7 were female. The ratio of male to female was 1.4:1. Age at presentation of GC ranged from 20 years to 76 years. The duration of symptoms before diagnosis was remarkably variable, ranging from 10 days to 2 years, and was aggravated sexual trends. Main symptoms: headache (8 cases), limb weakness (6 cases), mental disorders and personality changes (6 cases), including the memory decline, intelligent diminish and unresponsive, epilepsy (n = 5), visual or vision changes (2 cases), unsteady gait (1 case), with one case of disturbance of consciousness (Case 4). Finding on neurological examination included memory decline, the reaction retardation or dementia (7 cases),papilloedema (7 cases), pyramidal tract impairment (8 cases), cranial nerve impairment (3 cases), decreased visual acuity (2 cases), ataxia (1 cases) and visual field defect (1 cases).All the patients underwent MRI on a Siemens 1.5 Tesla machine. The following sequences were performed: T2-weighted sequences, and gradientecho T1-weighted sequences before and after gadolinium injection. In 8 patients, CT was also performed. CT/MRI-guided stereotactic biopsies were performed in all the 17 cases. All the procedures were done under local anesthesia with Leksell stereotactic system. The"four point-one line"hand count method was used to determine the coordinate in 3 cases and the Computer-assisted Planning for Neurosurgery (CAPN) in 14 cases. The specimens obtained from stereotactic biopsy were conventional fixed, HE staining and observed using electron microscopic. The therapeutic regimens did not follow a fixed protocol. Of the 17 cases, 2 cases declined chemotherapy and radiotherapy and only received supportive treatment. 2 cases received Robot-assisted stereotactic techniques permanent I125 seed implantation within the interstitial radiotherapy. 4 cases completed the whole course of radiotherapy and received the planned dose of between 44.0 and 57.6 Gy. The field of irradiation was whole brain. 3 cases received chemotherapy consisting of either PCV (procarbazine, 60 mg/m2 on days 8 to 21; CCNU, 110 mg/m2 on day 1; and vincristine, 1.4 mg/m2 on days 8 and 29) or temozolomide (TMZ; 150 to 200 mg/m2 for 5 days every 4 weeks). The remaining 6 cases received whole brain radiation therapy combined with chemotherapy. The chemotherapy was carried out in 4 to 6 weeks after the end of the radiotherapy.Results: In all patients, radiological examination showed an diffuse, poorly defined lesion in the brain, with a variable degree of swelling of the involved area. The extent of lesion involvement is as follows: 3 areas in 5 cases, 4 areas in 2 cases, 5 areas in 3 cases, 6 areas in 6 cases, 7 areas in 1 cases. Enlargement of the corpus callosum was detected in 10 cases and involvement of the brain stem in 3. In 8 cases, the basal ganglia, thalami and hypothalamus were involved. Iso-to hypodense lesions are seen on CT with a more or less diffuse mass effect with no enhancement after intravenous contrast medium administration. The MRI studies showed a low signal on T1-weighte image and a high signal on T2-weighed images in the regions of tumoral infiltration with a loss of differentiation between gray and white matter. Contrast enhancement was only seen in 4 cases. Mass effect was absent or minimal. There was no signal of necrosis and hemorrhage. MRS reveals that increased choline(Cho)/creatine(Cr) and choline(Cho)/ N- acetylaspartate (NAA) ratios in tumors as compared with normal brain tissue. Histological examination reveals the widespread infiltration of neoplastic glial cells with minimal destruction of pre-existing structures, frequently a preferential infiltration of the white matter. All the cases are astrocytomas , the grade are as follow: 3 case of gradeâ… , 8 cases of gradeâ…¡, 2 case of gradeâ…¡-â…¢,4 cases of gradeâ…¢. The overall median survival time is 9 months (range from 21 days to 24 months). The median lengths of survival are as follow: supportive treatment, 44.5 days; 125I seeds brachytherapy, 13.5 months; radiotherapy alone, 11.0 months; chemotherapy alone, 7 months; radiotherapy followed by chemotherapy, 17.5 months. Survival was not significantly different (P>0.05, log-rank test) between 7 cases received WBRT or chemotherapy alone and 6 cases received WBRT combined chemotherapy.Conclusion: 1. Patients with GC most present with an insidiously evolving syndrome. Clinical findings for patients with GC are usually complex, mainly determined by the anatomic site involved. 2. Radiological examination shows that lesions infiltrate more than two lobes, involving mainly the white matter, often corpus callosum, thalamus, brain stem. Iso-to hypodense lesions are seen on CT. The MRI studies showed a low signal on T1-weighte image and a high signal on T2-weighed images. Contrast enhancement is absent or minimal. Hemorrhage and necrosis is seldom. 3. Histological feature of GC is the widespread infiltration of neoplastic glial cells with minimal destruction of pre-existing structures, Hemorrhage and necrosis is seldom. 4. When patients have symptoms such as headache, personality changes, or focal neurological deficits, and when neuroimaging provides MRI evidence of a diffuse infiltrative process involving more than two cerebral lobes and often extending to infratentorial structures, a diagnosis of GC should be considered. The definite diagnosis should be confirmed by histological examination. Stereotactic biopsy is a powerful and safe method to provide tissue diagnosis of GC. 5. Surgery is not a visible option for GC, Radiotherapy, chemotherapy and seeds brachytherapy seem to be helpful for GC. However; the median survival is short and the prognosis is firmly grim.
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