Primary central nervous system lymphoma in the United States, 1975–2017

Lv, Chenglan; Wang, Jing; Zhou, Min; Xu, Jingyan; Chen, Bing; Wan, Yuan

doi:10.1177/20406207211066166

Cited by 21 publications

(10 citation statements)

References 42 publications

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“…Primary intraocular lymphoma (PIOL) is a rare heterogeneous malignancy and considered a subset of primary central nervous system lymphoma (PCNSL), with lymphoma cells initially existing only in the eyes [1,2]. As PIOL is a rare subset of PCNSL, most of its epidemiology data are deduced from studies on PCNSL, whose incidence rate has increased fivefold over the past 40 years, with its peak incidence occurring in those aged 75-84 years [3][4][5]. Between 15 to 25% of PCNSL patients have or will eventually develop ocular lymphoma [5].…”

Section: Introductionmentioning

confidence: 99%

Epidemiology and survival outcomes of patients with primary intraocular lymphoma: a population-based analysis

Zhang

Chen

et al. 2022

BMC Ophthalmol

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Background Primary intraocular lymphoma (PIOL) is a rare malignancy with a poor prognosis, but its optimal therapy remains unclear. Herein, we aimed to analyze the epidemiology and survival outcomes of PIOL patients based on a population-based cancer registry in the United States. Methods Patients diagnosed with PIOL between 1992 and 2018 were identified from the Surveillance Epidemiology and End Results program. The patients were divided into two groups: those aged < 60 years and ≥ 60 years. We used the chi-squared test to analyze the differences between the two groups. Descriptive analyses were performed to analyze epidemiological characteristics and treatment. The likely prognostic factors were analyzed by Kaplan–Meier curves and Cox proportional hazards models. Results The overall incidence of PIOL was 0.23/1,000,000, which was steadily increasing from 1992 to 2018, with an annual percentage change of 2.35. In total, 326 patients (mean age, 66.1 years) with PIOL were included in this study, 72.1% were aged ≥ 60 years, 84.4% were White, and 60.4% were female. The most common pathological type was diffuse large B-cell lymphoma (DLBCL), but in patients aged < 60 years, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue was the most common. The disease-specific survival rates were 74.2% and 61.5% 5 and 10 years after diagnosis, respectively. Survival analysis found that surgery, radiation, and chemotherapy did not lead to better prognosis. Conclusions PIOL is a rare disease with poor prognosis, and its incidence has been increasing for nearly 30 years. It usually affects people aged ≥ 60 years, and DLBCL is the most common pathological type of PIOL. Patients aged < 60 years and with non-DLBCL type have improved survival. Survival of PIOL has improved in recent years.

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Section: Introductionmentioning

confidence: 99%

Epidemiology and survival outcomes of patients with primary intraocular lymphoma: a population-based analysis

Zhang

Chen

et al. 2022

BMC Ophthalmol

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“…It has been shown that the 5-year survival rate of lymphomas in the ileocecal region was found to be 64.3% which is higher than that in large intestine (48.8%) and small intestine (32.5%) [17]. Primary central nervous system lymphoma on the other hand showed a low 5-year survival rate of 18% [18]. at being said, appendicular lymphomas have a good prognosis as it was shown that low-grade tumors including lymphomas have a 5-year survival rate of 67%-97% [19].…”

Section: Discussionmentioning

confidence: 96%

Double-Expressor Appendiceal Burkitt’s Lymphoma: A Case Report and Literature Review

Dukmak

Abugharbieh

Emar

et al. 2022

Case Reports in Hematology

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Background. Appendiceal lymphoma is a very rare entity accounting for 0.015% of all gastrointestinal lymphoma cases. Acute appendicitis is the most common presentation of primary appendix neoplasms. Burkitt’s lymphoma presenting as an acute appendicitis is a rare entity with around 21% of the cases presenting as a lower iliac fossa mass. Case Presentation. A 23-year-old male was admitted to the surgical ward as a case of acute appendicitis with localized tenderness in the right iliac fossa, positive rebound tenderness, a positive Rovsing’s sign, and ultrasound findings of suspected complicated appendicitis. Appendectomy was performed. Histopathological examination of the appendectomy specimen revealed a double-expressor non-Hodgkin diffuse large cell lymphoma with Burkitt’s-like morphology. He was sent for chemotherapy treatment. Conclusion. Only 34 cases of Burkitt’s lymphoma have been reported to present as acute appendicitis. Histological examination following appendectomy for an apparent appendicitis is essential. Furthermore, complete blood count and a computed tomography scan aid the diagnosis of lymphoma. Double-expressor lymphoma has been shown to have poor outcomes. Therefore, prompt and aggressive treatment is vital.

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“…2 In recent years PCNSL has shown a notable increase in incidence, reaching 0.5 per 100,000 people in immunocompetent patients. 3 Over the last three decades there has been signi cant progress in treatments and prognosis due to insightful retrospective series, single-arm phase II trials, and a set of randomized trials that have collectively contributed to the establishment of treatment guidelines. 4 Another even more rare entity is lymphoma con ned to the blood vessels within the CNS, intravascular lymphoma (IVL).…”

Section: Introductionmentioning

confidence: 99%

Primary CNS Lymphoma: age as a prognostic biomarker

Ohanyan,

Buxbaum,

Stein

et al. 2024

Preprint

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Background Lymphomatous involvement of the CNS incidence has been increasing in recent years. However, the rarity of these diseases results in a scarcity of available data regarding their clinical presentation natural history and prognosis. We aimed to investigate neurological characteristics of uncommon lymphomatous involvements confined to the central nervous system (CNS) and identify key variables that could serve as predictive biomarkers for treatment outcomes. Methods We searched for patients presenting with neurological symptoms and diagnosed with CNS-restricted lymphomatous involvement between 2005 and 2023. Results We identified 44 cases (41 with primary central nervous system lymphoma diagnosis, 93%; 3 intravascular lymphoma 7%). Overall median time from the onset of symptoms onset to diagnosis was 47 days: range (6-573 days) and was not statistically different comparing patients > 60 years old, p = 0.22. The median follow-up time was 1144 days (27-3501 days). Cognitive deterioration was the most common presenting symptom in 19/44 (43%). Brain MRI revealed lobar lesions as the most frequent location of pathology (24/44, 55%). Death was reported in 30 patients (68%) by the end of the study period with a median survival of 666 days; (median range 17-3291 days) and was significantly more common in the group experiencing relapses p = 0.04(CI: 0.99,0.03). Patients with relapses had a 4 times higher chance of death p = 0.04 (95% CI: 0.03–0.99) HR = 4.1 (CI 1.01–16.09). Time to diagnosis was associated with higher mortality, p = 0.04 (95% CI: 0.02–0.97) and survival p = 0.02 (95% CI: 0.005–0.54) as well with Eastern Cooperative Oncology Group at the last follow-up p = 0.006 (95% CI: 0.0012–0.62). Conclusions Patients had significant delays in diagnosis, affecting patient outcomes. Cognitive deterioration and lobar lesions were prominent clinical and radiological features. Mortality was notably higher in older patients, relapses, and a longer time to diagnosis.

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Primary central nervous system lymphoma in the United States, 1975–2017

Cited by 21 publications

References 42 publications

Epidemiology and survival outcomes of patients with primary intraocular lymphoma: a population-based analysis

Epidemiology and survival outcomes of patients with primary intraocular lymphoma: a population-based analysis

Double-Expressor Appendiceal Burkitt’s Lymphoma: A Case Report and Literature Review

Primary CNS Lymphoma: age as a prognostic biomarker

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