RESUMO
PURPOSE: Untethering surgery for lumbosacral lipoma is a preventive procedure, and avoidance of complications and good long-term outcomes are required. We introduced presurgical interactive virtual simulation (IVS) applying three-dimensional multifusion images using a haptic device aimed at improving operative outcomes. METHODS: Fourteen patients with newly diagnosed lumbosacral lipoma were recruited and underwent preoperative IVS. The median age at surgery was 8 months. A three-dimensional image analysis system was used to extract and fuse structures necessary for surgery, such as the lipoma, spinal cord and skin, from CT and MRI, and create three-dimensional multifusion images. The created images were individually converted to standard triangulated language format and loaded onto a workstation (Geomagic freeform™) that could be freely transformed, and the laminectomy range and lipoma extraction procedure were examined. Presurgical IVS was performed, and the actual surgery was performed. RESULTS: The disease types were dorsal, caudal, lipomyelomeningocele, transitional, and filum in 5, 5, 2, 1, and 1 patients, respectively. The surgical procedure and extent of the laminectomy were as planned for all patients. Resection of the lipomas tended to be less than expected preoperatively because of positive reactions on intraoperative monitoring. No postoperative complications were observed. The median postoperative follow-up period was 29 months, and there were no reoperations during the observation period. CONCLUSIONS: Although there are various types of lumbosacral lipoma, surgery can be safely performed by performing presurgical IVS. The short-term course is good; however, long-term follow-up is necessary for the appearance of neurological symptoms associated with growth and re-tethering.
Assuntos
Lipoma , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Humanos , Lactente , Neoplasias da Medula Espinal/cirurgia , Resultado do Tratamento , Imageamento Tridimensional , Interface Háptica , Neoplasias da Coluna Vertebral/cirurgia , Lipoma/cirurgia , Região Lombossacral/cirurgiaRESUMO
OBJECTIVE: Management of cervicomedullary compression due to foramen magnum stenosis in achondroplasia remains controversial, especially for patients with no symptoms or mild symptoms. We examined the effectiveness of polysomnography (PSG) as an indicator for cervicomedullary decompression treatment. METHODS: We retrospectively reviewed nine achondroplasia cases (mean age 1 year and 9 months) treated from 2008 to 2015. All patients were examined by PSG, magnetic resonance imaging (MRI), and otolaryngeal fibroscopy. We analyzed demographic data, clinical presentation, degree and type of respiratory impairment, severity of foramen magnum stenosis and concomitant cervicomedullary compression, treatment (conservative or surgical), and clinical outcome. RESULTS: Eight of nine patients presented with no severe symptoms in the daytime. However, MRI revealed four severe, four moderate, and one mild case of cervicomedullary compression, and PSG demonstrated severe sleep apnea in four cases and moderate sleep apnea in five cases. All sleep apnea cases were obstructive or obstructive-dominant. Fibroscopy revealed no upper airway stenosis in six cases and mild stenosis in three cases. Four patients who had severe sleep-related respiratory disturbance on PSG and severe or moderate cervicomedullary compression were treated by cervicomedullary decompression. Three of these patients demonstrated improved sleep respiration soon after surgery, while one required temporary tracheostomy due to bilateral vocal cord paralysis caused by compression during intratracheal intubation. CONCLUSION: Polysomnography can be a useful indicator for cervicomedullary decompression surgery, especially in cases of seemingly asymptomatic achondroplasia with severe foramen magnum stenosis.
Assuntos
Acondroplasia/complicações , Forame Magno/patologia , Forame Magno/cirurgia , Síndromes da Apneia do Sono/etiologia , Compressão da Medula Espinal/cirurgia , Pré-Escolar , Constrição Patológica/etiologia , Constrição Patológica/cirurgia , Descompressão Cirúrgica/métodos , Feminino , Humanos , Lactente , Masculino , Polissonografia , Estudos Retrospectivos , Síndromes da Apneia do Sono/diagnóstico , Compressão da Medula Espinal/etiologiaRESUMO
We present a pediatric case of a rapidly expanding third ventricle germ cell tumor (GCT). A 14-year-old boy suffered from gradual-onset central diabetes insipidus (DI) and received desmopressin treatment. Magnetic resonance imaging (MRI) showed nonspecific findings of the pituitary-hypothalamic axis. Nine months after the initial DI diagnosis, he developed progressively worsening headache. MRI demonstrated a third ventricle tumor causing noncommunicating hydrocephalus, although an MRI 16 weeks before admission did not show the lesion. We performed gross total resection (GTR) of the tumor in 2 stages: a translamina terminalis approach and an extended transsphenoidal approach. The lesion was histologically diagnosed as immature teratoma with some germinoma. His noncommunicating hydrocephalus resolved after surgery. Through postoperative radiochemotherapy (whole ventricle: 23.4 Gy/13 fractions, tumor bed: 27.0 Gy/15 fractions, and 3 courses of carboplatin-etoposide), he has was in complete remission at the 3-year follow-up and has continued his high school program. This case suggests the following: (1) a mixed GCT originating from the neurohypophysis/infundibulum can show rapidly expansive growth in a child with central DI; (2) GTR and adjuvant radiochemotherapy can result in a good therapeutic outcome in rapidly expanding GCT; and (3) the extended transsphenoidal approach is a complementary approach to transcranial resection of anterior third ventricle GCTs.
Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Hipófise/diagnóstico por imagem , Terceiro Ventrículo/diagnóstico por imagem , Adolescente , Neoplasias Encefálicas/cirurgia , Humanos , Masculino , Invasividade Neoplásica/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/cirurgia , Hipófise/cirurgia , Terceiro Ventrículo/cirurgiaRESUMO
OBJECTIVE: The objective of this study is to assess the prevalence and natural history of odontoid calcification and to identify factors related to its progression to crowned dens syndrome (CDS). METHODS: We reviewed a consecutive series of patients who underwent head CT upon admission to the neurosurgical ward of Niigata Prefectural Shibata Hospital. We evaluated the relationship between the presence of odontoid calcification and the patient's age, sex, and primary disorder for admission. The incidence of CDS was also determined, and factors related to the progression of odontoid calcification to CDS were analyzed. RESULTS: Odontoid calcifications were found in 88 out of 554 patients (15.9%) undergoing CT scans. Age, female, and stroke were predictive of odontoid calcification. The odds ratios (95% CIs) for age, female, and stroke estimated from the logistic analysis were 1.084 (1.054-1.114; p < .0001), 1.746 (1.06-2.875; p = .029), and 1.909 (1.123-3.247; p = .017), respectively. Eleven (12.5%) of the 88 patients with odontoid calcification developed CDS. The age, sex, and primary disorder for admission were not associated with the onset of CDS. CONCLUSIONS: Odontoid calcification is observed incidentally on head CT. Clinicians should keep in mind that patients with odontoid calcification might develop CDS, even if they are asymptomatic at the time of CT examination.
Assuntos
Calcinose/epidemiologia , Cervicalgia/epidemiologia , Processo Odontoide/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Calcinose/diagnóstico por imagem , Feminino , Humanos , Incidência , Masculino , Cervicalgia/diagnóstico por imagem , Prevalência , Tomografia Computadorizada por Raios XRESUMO
Objective: In this study, we provide long-term outcome data of patients with primary central nervous system lymphoma. Methods: The long-term outcomes of PCNSL patients diagnosed between 1982 and 2006 were reviewed. Neurological late neurotoxicity symptoms, neuroradiological brain atrophy and leukoencephalopathy were evaluated. Surviving patients completed the Quality of Life Questionnaire-30 and Brain Cancer Module-20. The differences in overall survival were assessed using the Kaplan-Meier method and log-rank test. The differences between groups in terms of each investigated parameter were analyzed using the Wilcoxon signed-rank test. Results: Among 112 PCNSL patients, there were 33 (29.4%) long-term (> 5 years) survivors. The median survival of all long-term survivors was 105.7 months; of these, 8 (7.1%) were alive at the latest follow-up, with a mean survival time of 170.2 months (range, 121.8286.4). Clinical assessment revealed severe neurotoxicity in 14 patients (42.4%), moderate neurotoxicity in 5 (15.1%), and normal status in 14 (42.4%). Correlations were seen between the neuroradiological imaging score changes and neurocognitive condition (P=0.0001), neurocognitive condition and the whole brain irradiation dose (P=0.0004), and atrophy and the whole brain irradiation dose (P=0.0035). Conclusions: A more severe clinical condition was found to be associated with increasing age and whole brain irradiation dose in long-term survivors with PCNSL.
Assuntos
Neoplasias do Sistema Nervoso Central/fisiopatologia , Linfoma não Hodgkin/fisiopatologia , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/mortalidade , Neoplasias do Sistema Nervoso Central/psicologia , Feminino , Humanos , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/psicologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Qualidade de Vida , SobreviventesRESUMO
Hemlock woolly adelgid, Adelges tsugae, is an invasive pest of hemlock trees (Tsuga) in eastern North America. We used 14 microsatellites and mitochondrial COI sequences to assess its worldwide genetic structure and reconstruct its colonization history. The resulting information about its life cycle, biogeography and host specialization could help predict invasion by insect herbivores. We identified eight endemic lineages of hemlock adelgids in central China, western China, Ulleung Island (South Korea), western North America, and two each in Taiwan and Japan, with the Japanese lineages specializing on different Tsuga species. Adelgid life cycles varied at local and continental scales with different sexual, obligately asexual and facultatively asexual lineages. Adelgids in western North America exhibited very high microsatellite heterozygosity, which suggests ancient asexuality. The earliest lineages diverged in Asia during Pleistocene glacial periods, as estimated using approximate Bayesian computation. Colonization of western North America was estimated to have occurred prior to the last glacial period by adelgids directly ancestral to those in southern Japan, perhaps carried by birds. The modern invasion from southern Japan to eastern North America caused an extreme genetic bottleneck with just two closely related clones detected throughout the introduced range. Both colonization events to North America involved host shifts to unrelated hemlock species. These results suggest that genetic diversity, host specialization and host phylogeny are not predictive of adelgid invasion. Monitoring non-native sentinel host trees and focusing on invasion pathways might be more effective methods of preventing invasion than making predictions using species traits or evolutionary history.
Assuntos
Genética Populacional , Hemípteros/genética , Cicutas (Apiáceas) , Espécies Introduzidas , Animais , Teorema de Bayes , DNA Mitocondrial/genética , Ásia Oriental , Genótipo , Herbivoria , Repetições de Microssatélites , América do Norte , Análise de Sequência de DNARESUMO
BACKGROUND: For pineal nongerminomatous malignant germ cell tumors (NGMGCTs), we mainly performed radical tumor resection during initial treatment combined with adjuvant therapy. METHODS: We retrospectively analyzed 17 patients treated for pineal NGMGCTs between 1986 and 2007 at the University of Niigata. RESULTS: Twelve patients underwent total or subtotal resection of their tumor via the occipital transtentorial approach. Five patients underwent partial resection, and four of them later underwent total resection by salvage surgery. After surgery, eight patients were treated with combined radiochemotherapy including whole-brain irradiation, two received radiation monotherapy, one had chemotherapy with local irradiation, and six were treated with chemotherapy alone. The median follow-up period for surviving patients was 179 months. The 10-year overall survival and progression-free survival rates for the radiochemotherapy group were both 75.0 % (two patients had a recurrence and died); the rates for other adjuvant therapies were 77.8 % (two died) and 22.2 % (seven had a recurrence), respectively. Radiochemotherapy was significantly associated with an increased rate of progression-free survival compared with the other adjuvant therapies (p = 0.0396). CONCLUSIONS: For pineal NGMGCTs, initial treatment strategies including gross total resection of the tumor before or after whole-brain irradiation and chemotherapy provided good therapeutic outcomes. Obtaining complete remission of the primary tumor, irrespective of the timing of surgical resection (i.e., before or after adjuvant therapies), or complete response by neoadjuvant radiochemotherapy during an initial treatment appears to be essential for improving therapeutic outcomes of intracranial NGMGCTs.
Assuntos
Neoplasias Embrionárias de Células Germinativas/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Pinealoma/cirurgia , Adolescente , Quimiorradioterapia , Quimioterapia Adjuvante , Criança , Feminino , Humanos , Masculino , Neoplasias Embrionárias de Células Germinativas/terapia , Glândula Pineal/cirurgia , Pinealoma/terapia , Adulto JovemRESUMO
Molecular analysis of the growing teratoma syndrome has not been extensively studied. Here, we report a 14-year-old boy with a growing mass during treatment for a mixed germ cell tumor of the pineal region. Tumor markers were negative; thus, growing teratoma syndrome was suspected. A radical resection via the occipital transtentorial approach was performed, and histopathological examination revealed a teratoma with malignant features. Methylation classifier analysis confirmed the diagnosis of teratoma, and DMRT1 loss and 12p gain were identified by copy number variation analysis, potentially elucidating the cause of growth and malignant transformation of the teratoma. The patient remains in remission after intense chemoradiation treatment as a high-risk germ cell tumor.
Assuntos
Teratoma , Humanos , Masculino , Teratoma/terapia , Teratoma/patologia , Adolescente , Neoplasias Encefálicas/terapia , Terapia CombinadaRESUMO
The present study aimed to identify genes associated with patient survival to improve our understanding of the underlying biology of gliomas. We investigated whether the expression of genes selected using random survival forests models could be used to define glioma subgroups more objectively than standard pathology. The RNA from 32 non-treated grade 4 gliomas were analyzed using the GeneChip Human Genome U133 Plus 2.0 Expression array (which contains approximately 47 000 genes). Twenty-five genes whose expressions were strongly and consistently related to patient survival were identified. The prognosis prediction score of these genes was most significant among several variables and survival analyses. The prognosis prediction score of three genes and age classifiers also revealed a strong prognostic value among grade 4 gliomas. These results were validated in an independent samples set (n = 488). Our method was effective for objectively classifying grade 4 gliomas and was a more accurate prognosis predictor than histological grading.
Assuntos
Glioblastoma/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise de Sequência com Séries de Oligonucleotídeos/métodos , Prognóstico , RNA/genética , Análise de Sobrevida , Transcriptoma , Adulto JovemRESUMO
BACKGROUND: Lesions associated with a thickened pituitary stalk (TPS lesions) revealed by magnetic resonance imaging have a diverse pathology. Accordingly, for clinical decision-making, it is necessary to make a diagnosis based on histopathological examination of the TPS lesions. The objectives of this study were to review endoscopic biopsies of TPS lesions and to assess the surgical strategy for treating these lesions. METHODS: Eleven patients (four males and seven females) aged from 6 to 75 years underwent endoscopic biopsy of a TPS lesion between 2006 and 2011 at University of Niigata. The relationships of the extent of lesions with surgical approaches were retrospectively examined. RESULTS: Among the 11 patients, a biopsy was performed via an endoscopic transsphenoidal approach for five with intrasellar lesions; via an endoscopic extended transsphenoidal approach for two with localized TPS lesions; and via an endoscopic intraventricular approach for four with the lesion protruding from the infundibulum. Histopathological examinations of all the lesions confirmed diagnoses of germinoma in four patients, hypophysitis in three, Langerhans cell histiocytosis in two, craniopharyngioma in one, and cancer metastasis in one. None of the 11 patients had further deterioration of pituitary function postoperatively. CONCLUSIONS: Endoscopic biopsy of TPS lesions is a less invasive alternative to open biopsy requiring transcranial surgery. The endoscopic transsphenoidal approach is most suitable for biopsies of TPS lesions associated with intrasellar lesions. Otherwise, the endoscopic intraventricular approach seems reasonable for intraventricular lesions protruding from the infundibulum, and the endoscopic extended transsphenoidal approach appears appropriate for localized TPS lesions.
Assuntos
Biópsia/métodos , Endoscopia , Doenças da Hipófise/patologia , Doenças da Hipófise/cirurgia , Hipófise/patologia , Adolescente , Adulto , Idoso , Criança , Craniofaringioma/patologia , Craniofaringioma/cirurgia , Feminino , Germinoma/patologia , Germinoma/cirurgia , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Hipófise/cirurgia , Estudos Retrospectivos , Adulto JovemRESUMO
The organic light-emitting diode (OLED) display delivers a bright and high-contrast image compared to the liquid crystal display. The first experience of neuroendoscopic surgery using an OLED display was reported and its stereoscopic effect emphasized.
Assuntos
Ventrículos Cerebrais/cirurgia , Luz , Iluminação/instrumentação , Neuroendoscopia/instrumentação , Compostos Orgânicos/química , HumanosRESUMO
Gall-forming aphid species Tetraneura nigriabdominalis and T. fusiformis and its closely related species are taxonomically revised. By referring to the original descriptions, the name T. nigriabdominalis (Sasaki, 1899) is discarded as an erroneous combination, and T. akinire Sasaki, 1904 is adopted as a valid name. The T. akinire species group is defined as having long claws in the first instar nymphs of the root generation. Of the T. akinire species group distributed in Korea and Japan, T. ovaliformis sp. nov., which induces globular galls on the leaves of Ulmus davidiana var. japonica, is described, and T. akinire and T. sorini Hille Ris Lambers, 1970 are redescribed. A molecular phylogeny based on partial sequences of mitochondrial cytochrome c oxidase subunit I (COI) indicates that T. akinire is composed of two clusters, one (type A) which is distributed widely from Europe to East Asia on Ulmus spp., and the other (type B) which is found in Hokkaido, northern Japan on U. davidiana var. japonica and in tropical regions as anholocyclic lineages. Tetraneura fusiformis Matsumura, 1917, which has often been treated as a junior synonym of T. nigriabdominalis, likely corresponds to type B. The taxonomic status of T. fusiformis is discussed and this species is tentatively considered as a junior synonym of T. akinire sensu novo.
Assuntos
Afídeos , Ulmus , Animais , Afídeos/genética , Ninfa , Filogenia , Folhas de PlantaRESUMO
Physical inactivity is one of the causes of most metabolic syndromes. The incidence of metabolic syndrome is expected to increase in the near future because of the reduced opportunities for exercise caused by COVID-19. Non-alcoholic fatty liver disease (NAFLD) is currently the most common cause of chronic liver disease. Changes in diet and lifestyle have led to a dramatic increase in the prevalence of NAFLD in the world. NAFLD is characterized by excessive triglyceride (TG) accumulation in the hepatocytes due to both increased inflow of free fatty acids and de novo hepatic lipogenesis. Thus far, no study quantitatively assessed the liver fat deposition after a rapid decline in physical activity. Herein, we describe a case of a 17-year-old Japanese boy with severe fat infiltration of the liver, due to a rapid decline in physical activity, treated at our facility. Our rehabilitation and nutritional support teams administered appropriate exercise and nutrition support to reduce weight and improve liver dysfunction. Our findings support dietary changes and exercise therapy to manage such cases.
Assuntos
Plexo Corióideo/patologia , Criptococose/patologia , Cryptococcus , Ventrículos Laterais/patologia , Síndromes de Compressão Nervosa/patologia , Antifúngicos/uso terapêutico , Plexo Corióideo/microbiologia , Plexo Corióideo/cirurgia , Criptococose/tratamento farmacológico , Criptococose/microbiologia , Feminino , Humanos , Ventrículos Laterais/microbiologia , Ventrículos Laterais/cirurgia , Pessoa de Meia-Idade , Síndromes de Compressão Nervosa/etiologia , Síndromes de Compressão Nervosa/cirurgia , Procedimentos NeurocirúrgicosRESUMO
Many studies have evaluated transmission abilities of laboratory-reared aphids for potato virus Y (PVY), but few have focused on PVY-harboring species of field-collected aphids and the strains of PVY harbored by aphids. In the present study, we collected alate aphids in yellow pan traps in potato fields with Japanese commercial cultivars in Hokkaido, northern Japan in single 24-h periods during the tuber bulking stage and examined whether individual whole aphids harbored PVY by nested RT-PCR. PVY-positive individuals were identified to species using the gene sequence for cytochrome c oxidase subunit I and, when needed, morphological data and distribution records. In addition, individual strains of PVY harbored were determined using partial sequences of coat protein. Among 1,857 aphids trapped, 195 aphids had PVY and comprised 19 species; 17 species were identified to species-group taxa. Most of the aphid species detected as PVY positive colonize weeds that are common around potato fields in Hokkaido. Five species-group taxa had not been reported previously as a vector aphid of PVY and might be new PVY-vector species. PVYNTN was most frequently detected from PVY-positive aphids as found recently in PVY-infected potatoes in commercial fields in Hokkaido. Two or three PVY strains were rarely detected from a single aphid, and no obvious difference was found in the proportion of the harbored PVY strains among positive aphid species. The first documentation of the species composition of PVY-harboring aphids and the strains of PVY harbored in East Asia should aid understanding of the epidemiology of PVY in Japan.
Assuntos
Afídeos/virologia , Insetos Vetores/virologia , Potyvirus/isolamento & purificação , Animais , JapãoRESUMO
Germ cell tumors typically occur in children and adolescents. We here report a rare case of de novo pineal region germinoma in the seventh decade of life. A 62-year-old man presented with double vision. Computerized tomography and magnetic resonance imaging (MRI) identified a heterogeneously enhanced tumor with calcification in the pineal region with ventricular dilatation due to aqueduct stenosis. The tumor had not been observed at all on MRI obtained 2 years previously. The patient underwent endoscopic biopsy and third ventriculostomy for the obstructive hydrocephalus. The tumor was histopathologically diagnosed as a pure germinoma. The patient underwent radiomonotherapy, resulting in his complete remission, which was confirmed by a series of follow-up MRI studies and hematological examinations. Intracranial germinoma should be considered in the differential diagnosis of pineal region tumors regardless of age, even though the tumor was undetectable on the previous neuroimaging.
RESUMO
BACKGROUND: Chemotherapy with or without radiotherapy is the mainstay of treatment for primary central nervous system lymphoma (PCNSL). High-dose methotrexate (MTX) is the most effective drug available to treat these lesions, either as a single agent or in combination with other drugs. Due to the lack of well-conducted randomized trials, the optimal treatment remains controversial. Available retrospective studies are difficult to discuss, however, some common themes can be found. METHODS: One hundred and twelve patients with PCNSL were treated with four different regimens over a period of 24 years. Treatment regimens were: whole-brain irradiation (WBI) alone, MVP (MTX, vincristine, and predonisolone), ProMACE-MOPP hybrid (cyclophosphamide, pirarubicin, etoposide, vincristine, procarbazine, prednisone, and MTX) and R-MTX (rituximab, MTX, pirarubicin, procarbazine, and prednisone) combined-modality therapy. RESULTS: The median failure-free survival was 16 months, and the median overall survival (OS) was 24 months. The 2- and 5-year actuarial probability of survival was 52.4 +/- 4.8% [95% confidence intervals (CI)] and 30.2 +/- 4.8% (95% CI), respectively. The ProMACE-MOPP protocol, Karnofsky performance status (KPS), MTX dose and WBI were associated with good OS by univariate models. By multivariate analysis, MTX dose, WBI dose, and its square dose were significantly associated with good OS. 20-30 Gy WB, and 500 mg/m(2) of MTX dose appeared important determinants of OS. CONCLUSIONS: A modest dose of MTX (500 mg/m(2)) followed by reduced-dose WBI for patients who respond appears a feasible treatment approach that minimizes serious toxicity.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/radioterapia , Irradiação Craniana , Linfoma/tratamento farmacológico , Linfoma/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Quimioterapia Adjuvante , Cognição/efeitos dos fármacos , Cognição/efeitos da radiação , Irradiação Craniana/efeitos adversos , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Humanos , Estimativa de Kaplan-Meier , Avaliação de Estado de Karnofsky , Leucovorina/administração & dosagem , Masculino , Mecloretamina/administração & dosagem , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prednisona/administração & dosagem , Procarbazina/administração & dosagem , Radioterapia Adjuvante , Estudos Retrospectivos , Terapia de Salvação/métodos , Resultado do Tratamento , Vincristina/administração & dosagemRESUMO
Craniosynostosis associated with Chiari malformation (CM) is usually found in infants with an underdeveloped posterior fossa. We here present a case of adult craniosynostosis, CM, and symptomatic syringomyelia caused by the protrusion of the posterior rim of the foramen magnum without a tight posterior fossa. A 22-year-old woman with an abnormal head shape and forearm hypesthesia was given a diagnosis of sagittal suture synostosis with CM and syringomyelia caused by foramen magnum stenosis. She underwent foramen magnum decompression with a C1 laminectomy without cranial vault expansion or duraplasty. Her symptoms and radiographical findings improved after surgery. In cases of non-operative craniosynostosis with CM, clinicians should be alert to late-onset syringomyelia and choose surgical strategies according to the pathophysiology.
RESUMO
We report the efficacy of salvage therapy with a modified ProMACE-MOPP combined with radiation in patients with primary central nervous system lymphoma (PCNSL). Thirty-two immunocompetent patients were treated with a regimen of pirarubicin, cyclophosphamide, etoposide, vincristin, and methotrexate (MTX: 500 mg/m(2)) administered in 21-day cycles. Patients received 20 Gy of whole-brain radiotherapy after three cycles of chemotherapy. A single cycle of chemotherapy was repeated every four months for two years. Nine patients with CNS relapse were retreated with additional cycles of the ProMACE-MOPP hybrid regimen with a 90% objective response rate. Median complete response (CR) duration was 13.2 months and median survival time (MST) for the nine patients treated after initial relapse was 30 months. One of 17 patients (5.8%) who had less than 20 Gy of whole brain irradiation developed dementia. In contrast, six of seven (85.7%) patients who had more than 30 Gy of whole brain radiotherapy became demented. Maintaining a moderate dose of MTX, while adding chemotherapeutic agents and 20 Gy of whole brain radiation therapy, improved disease control and overall survival and lowered the incidence of delayed neurologic toxicity in patients with PCNSL. Additional treatment with a ProMACE-MOPP hybrid regimen is still effective for relapsed disease.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/radioterapia , Linfoma/tratamento farmacológico , Linfoma/radioterapia , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/radioterapia , Terapia de Salvação/métodos , Adulto , Idoso , Neoplasias do Sistema Nervoso Central/mortalidade , Terapia Combinada , Ciclofosfamida/efeitos adversos , Ciclofosfamida/uso terapêutico , Doxorrubicina/administração & dosagem , Doxorrubicina/efeitos adversos , Doxorrubicina/análogos & derivados , Doxorrubicina/uso terapêutico , Etoposídeo/efeitos adversos , Etoposídeo/uso terapêutico , Feminino , Humanos , Linfoma/mortalidade , Masculino , Mecloretamina/efeitos adversos , Mecloretamina/uso terapêutico , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Síndromes Neurotóxicas/tratamento farmacológico , Síndromes Neurotóxicas/radioterapia , Prednisona/efeitos adversos , Prednisona/uso terapêutico , Procarbazina/efeitos adversos , Procarbazina/uso terapêutico , Estudos Retrospectivos , Terapia de Salvação/efeitos adversos , Análise de Sobrevida , Vincristina/efeitos adversos , Vincristina/uso terapêuticoRESUMO
We report a case of solitary fibrous tumor (SFT) in the cerebral convexity, and present characteristic radiological and surgical findings to determine its origin. The patient was a 59-year-old man with mental dullness and mild gait disturbance. CT scan and MR images showed a highly enhanced large mass lesion mimicking a meningioma in the left parietal convexity. However, neither dural enhancement nor tail sign indicative of meningioma was observed. Angiography showed prominent feedings from branches of the internal carotid and basilar arteries rather than the external carotid artery. For this reason, presurgically, we suggested hemangiopericytoma or other specific meningiomas as a differential diagnoses. Surgery confirmed that the tumor had no attachment to the dura mater and was covered by the arachnoid membrane. The bottom of the tumor adhered tightly to brain tissue. The origin was considered to be the brain surface, pia mater or a part of the arachnoid membrane. Histopathologically, the tumor was diagnosed as a SFT with findings of "attemless pattern" and diffuse CD34 staining. The radiological and surgical findings of the present case indicated in the cerebral convexity as a unique site of origin of SFT.