Incidence Of Primary Central Nervous System Lymphoma Research Articles

No Association between Polyomaviruses and Primary Central Nervous System Lymphomas of HIV-Seronegative and HIV-Positive Patients

No Association between Polyomaviruses and Primary Central Nervous System Lymphomas of HIV-Seronegative and HIV-Positive Patients

Incidence, clinical features, treatment and outcome of primary central nervous system lymphoma in NorwayA ten-year national survey

The incidence of primary central nervous system lymphoma (PCNSL) has been reported to increase in some parts of the world, while being stable in other regions. In an attempt to characterize the incidence rate, clinical features, treatment, outcome, and prognostic factors of PCNSL in Norway, we report our experience in a large unselected series of patients. Clinical features, histological diagnosis, radiological findings, treatment, and outcome of all patients diagnosed with PCNSL in Norway in the years 1989–1998 were registered. During the 10-year period 58 new cases of histologically verified PCNSL were registered in Norway. The annual incidence rate of PCNSL was on average 1.34 cases per million people with a non-significant increasing trend (p=0.069). For patients diagnosed before death (n=45) the estimated survival following histological diagnosis was 55%, 47%, and 23% at 1, 2, and 5 years, respectively. In Cox-regression analysis age, WHO performance status and treatment had independent prognostic impact on survival. In the studied decade, there was a non-significant trend towards increased incidence of PCNSL, perhaps due to increased availability of diagnostic imaging, especially magnetic resonance imaging.

Linfomas primarios del sistema nervioso central en pacientes con sida

Primary lymphoma is the most common neoplasm of the central nervous system (CNS) in AIDS patients. We retrospectively reviewed the clinical manifestations, neuroimaging findings, diagnostic methods used, histological characteristics, detection of Epstein-Barr virus (EBV) DNA by PCR in cerebrospinal fluid (CSF) and brain smears, and outcome of 18 HIV/AIDS patients with primary CNS lymphoma. The overall incidence of primary CNS lymphoma was 2.6%. Fifteen were men and mean age was 33.6 years. The most frequent clinical findings were focal neurological deficits and seizures. The mean CD4 T cell count at the time of diagnosis was 44 cells/microl. Primary CNS lymphoma presented as single, large (> 2.5 cm) lesions in 14 patients (77.8%). All the lesions were associated with a mass effect and surrounding edema. EBV DNA was detected in nine brain smears. In seven of these nine cases, EBV DNA was also found in CSF by PCR. Median survival after specific diagnosis was 75 days. This study upholds a link between EBV and these tumors. Primary CNS lymphoma was associated with a poor prognosis and short survival in this cohort of patients.

The continuing increase in the incidence of primary central nervous system non-Hodgkin lymphoma: a surveillance, epidemiology, and end results analysis.

Primary central nervous system lymphoma (PCNSL) is an extranodal form of non-Hodgkin lymphoma arising in the craniospinal axis. The incidence of PCNSL appears to be increasing. PCNSL incidence data from 1973-1997 were obtained from the nine Surveillance, Epidemiology and End Results (SEER) registries. To limit the influence of the human immunodeficiency virus on incidence rates, data of never-married males and females and persons of unknown marital status were excluded. As a surrogate for new technology, SEER data were reviewed by dates of diagnosis (surrogate for imaging) and compared with glioma incidence (surrogate for stereotactic neurosurgery and improved diagnostic neuropathology). Age-adjusted incidence rates were estimated and compared for the period prior to computed tomography (CT) (1973-1984) and the magnetic resonance imaging (MRI) period (1985-1997). The estimated annual percent change was calculated based on linear regression analyses using SEER*STAT. The incidence of PCNSL appears to be increasing in all SEER registries examined. All age groups demonstrated an increase over time. This increase was observed both in the CT era as well as in the MRI era. PCNSL age-adjusted incidence (0.15 to 0.48, a 3-fold increase) outpaced that of systemic lymphoma (14.1 to 18.5, a 33% increase) for the same registries over the same time periods. The rate of increase has begun to slow since 1985; the estimated annual percent change for PCNSL was three-fold higher during the period 1973-1985 compared with 1986-1997. The incidence rate of PCNSL continues to rise. The increase is evident in all age groups and in both genders. Data from the current study suggest that improved diagnostic tools, such as CT or MRI, cannot explain this increase.

Primary central nervous system lymphomas: incidence and survival in the Southern and Eastern Netherlands.

An excessive increase in the incidence of primary central nervous system lymphoma (PCNSL) has been reported since the mid-1980s in the U.S. and U.K. Clinical studies have shown that radiotherapy and chemotherapy may prolong survival. In the current study, the authors describe the incidence, treatment, and survival of an unselected group of patients with PCNSL in the southern and eastern Netherlands. Data regarding patients diagnosed between 1989-1994 were obtained from 4 population-based regional cancer registries in the southern and eastern Netherlands (n = 86) and the Eindhoven Cancer Registry for 1980-1988 (n = 6). Lymphomas were registered as PCNSL when a tissue diagnosis of CNS lymphoma was established for a patient with neurologic symptoms (i.e., lymphomas were not necessarily restricted to the CNS at the time of diagnosis). Only patients diagnosed during their lifetime with Stage I disease, Stage "IV" disease (i.e., diffuse CNS lymphoma), or disease of unknown stage were included (63 patients, 8 patients, and 15 patients, respectively, between 1989-1994). For 80 patients (93%) follow-up was complete until January 1, 1997. Between 1989-1994, an average World Standardized Rate of 2.3 cases and 1.7 cases per 1 million person-years, respectively, was reported for males and females. The median age of the patients at the time of diagnosis was 62 years, and was 66 years for patients with an unknown disease stage. In the area of the Eindhoven Cancer Registry the occurrence of PCNSL more than doubled from < 2% of all histologically confirmed primary CNS malignancies diagnosed between 1980-1985 to approximately 4% of cases diagnosed between 1986-1994. The median survival of all the patients was 4.1 months; the median survival was 5.8 months for patients with limited (Stage I and Stage IV) disease and was 0.6 months for patients with an unknown stage of disease. Approximately 65% of the patients with limited disease received radiotherapy and approximately 35% of such patients received chemotherapy. Furthermore, chemotherapy was given more often to patients age < 60 years who tended to have a slightly better survival than patients age > or = 60 years. The increase in the incidence of PCNSL in the 1980s may be explained in large part by changes in diagnostics and registration. The relatively high incidence and low survival rate of PCNSL in the southern and eastern Netherlands reported in the 1990s may be due in part to the inclusion of patients with systemic lymphoma and immunodeficiency disorders. However, a significant improvement in the prognosis of patients with PCNSL in the southern and eastern Netherlands diagnosed in the 1990s is unlikely.

Molecular analysis of primary central nervous system and primary intraocular lymphomas.

Primary central nervous system lymphoma (PCNSL) is usually a large B-cell, high grade non-Hodgkin's lymphoma (NHL) classified as a diffuse large cell lymphoma (DLCL). In rare cases, however, T cell lymphomas have been described. Although a relatively rare tumor, the incidence of PCNSL has increased dramatically over the past 15 years in both immuno-competent and immunocompromised patients. The disease is aggressive with a 5-year survival rate of less than 25 %. The cause of death is progressive and recurrent disease in the CNS, despite aggressive treatment. Approximately 20-25% of patients with PCNSL also have primary intra-ocular lymphoma (PIOL). PCNSL and PIOL are closely related and inter-connected pathologies involving two immune privileged sites. The study of PCNSL and PIOL has been limited due to the fact that viable malignant cells are rare and difficult to recognize. Moreover, the cells are difficult to culture and to date there is no good animal model for the disease. Here, we will present the current literature on the disease. In particular, we will present data suggesting that PCNSL in immuno-compromised and AIDS patients may correspond to two different pathologies. Furthermore, we will discuss how the study of these lymphomas can benefit from new advanced molecular biology techniques including single cell PCR and laser capture microdissection (LCM). PCNSL and PIOL are aggressive tumors, therefore, early diagnosis and prompt, aggressive treatment may improve prognosis. Advanced molecular biology will help delineate the oncogenesis of PCNSL and PIOL.

Caractéristiques épidémiologiques et évolution de l'incidence des lymphomes cérébraux primitifs observés chez 80 patients infectés par le VIH entre 1983 et 1999

Objective – To describe the epidemiological characteristics of primary central nervous system lymphoma (PCNSL) and the evolution of the incidence of this lymphoma in HIV-infected patients with a more than 17-year follow-up. Results – Eighty cases of PCNSL were analyzed from a data base of 2 263 AIDS subjects followed from 1983 to 1999 (3.5% of the patients with AIDS). At the time of diagnosis, PCNSL was the first AIDS defining event in 36% of the cases, median CD4 count was 9/mm 3 (0–134); 82% of the patients were given antiretroviral therapy (HAART = 0). Only eight cases of PCNSL were observed after 1996 (median HIV RNA level: 250 000 copies/mL (24 000–1 500 000)). The incidence was 39 per 1 00 patients-year in 1991 and decreased to 1.9 in 1999. At the end of the study, 78 patients had died (98%). The median survival was one month before 1996 ([0–27], n=72), and was ten months after ([0–44], n=8). Two patients were still alive 38 and 44 months after diagnosis. After 1996, survival was increased in patients with good response to antiretroviral treatment and in patients with high CD4 count at the moment of diagnosis. Conclusion – After the introduction of HAART (1996), the incidence of PCNSL has decreased drastically and survival was increased.

Primary central nervous system lymphoma - the royal melbourne hospital experience 1986-1991

Eight cases of primary central nervous system (CNS) lymphoma were identified between June 1987 and April 1991. These eight cases of primary CNS lymphoma represent 1.8% of primary CNS neoplasms undergoing neurosurgery at the Royal Melbourne Hospital during this period. The patients presented with symptoms ranging from focal seizures and focal neurological deficits to symptoms of raised intracranial pressure. The mean duration from onset of symptoms to presentation was 14 weeks. There was an equal male: female incidence. Patient age ranged from 28yrs to 70yrs (mean 51, median 44). The anatomical distribution was parietal lobe in three, frontal lobe in two, and basal ganglia, Cauda equina and cerebellar-pontine angle, one each. A single lesion only was present in six cases while in two, multiple lesions could be defined by CT scanning. CT scanning showed low attenuation areas with contrast enhancement in four cases. All patients were HIV seronegative. By the International Working Formulation three cases were high grade, three intermediate grade and two were not classified. Six tumours underwent immunophenotyping with a range of B-cell, T-cell and monocyte-macrophage markers. All six were found to express markers of B-cell lineage. Our incidence of primary CNS lymphoma is higher than previous reports, and indicate that primary CNS lymphoma is usually of B-cell lineage, occurs most frequently in the parietal lobe, and is usually of high grade (International Working Formulation).