Long-term Survival Following Extra-neural Metastasis From a Pineoblastoma

Primary central nervous system lymphoma (PCNSL) is a rare disease with a poor prognosis. It usually remains confined to central nervous system (CNS). Reports of metastases outside of the CNS are rare. We report a patient with well-documented PCNSL who responded to treatment, but subsequently developed a histologically confirmed subcutaneous metastasis to the left leg without local failure.     

Primary central nervous system lymphoma as a secondary malignancy

Primary central nervous system lymphoma (PCNSL) is a rare neoplasm, but it is occurring with increased frequency even among apparently immunocompetent patients. Although secondary malignancies frequently involve the lymphoreticular system, PCNSL has been reported as a second neoplasm only once previously. Seven patients are discussed who developed PCNSL after successful treatment for a prior neoplasm. The original cancer was colon (one), breast (one), thyroid (one), Hodgkin's disease (two), and non-Hodgkin's lymphoma (two). Patients with systemic non-Hodgkin's lymphoma were thought to have a separate cerebral lymphoma on the basis of a prolonged disease-free interval from their systemic lymphoma, and the absence of systemic disease, when PCNSL was diagnosed and through subsequent follow-up. The PCNSL developed a median of 10 years after the diagnosis of the first tumor and 6 years after the last evidence of systemic disease. The diagnosis of PCNSL was often delayed because of confusion with brain metastases, and initial shrinkage or disappearance of the lesion after corticosteroids. Formation of PCNSL may be a consequence of treatment for the first malignancy, reflect an unidentified inherent predisposition to neoplastic transformation, or result from the changing epidemiology of PCNSL in the general population. These mechanisms are not mutually exclusive, and a single hypothesis cannot account for all these cases.     

A case with coincidental diagnosis of primary central nervous system lymphoma and lymph node sarcoidosis

Primary central nervous system lymphoma (PCNSL) is rare. Clinical and histological differential diagnosis of systemic lymphoma and sarcoidosis continues to be a challenge. The first case report in the German and English literature of PCNSL and synchronous sarcoidosis is presented. Synchronous mediastinal lymphadenopathy suggestive of non-Hodgkin’s lymphoma (NHL) or sarcoidosis was noted. Both conditions require alternative therapeutic and prognostic considerations to PCNSL. A regime of intrathecal and adjuvant systemic chemotherapy led to transient clinical improvement prior to the patient’s demise through overwhelming sepsis and multiorgan failure. Post mortem findings confirmed synchronous PCNSL with mediastinal lymph node sarcoidosis.     

Unusual variants of primary central nervous system lymphoma

Rare variants of primary central nervous system lymphoma (PCNSL) include unusual sites of presentation (eg, neurolymphomatosis and primary leptomeningeal lymphoma) and uncommon pathologic entities. Neurolymphomatosis involves peripheral nerves and nerve roots in addition to systemic and central nervous system (CNS) sites. Diagnosis requires a high index of suspicion, and treatment incorporates the principles of therapy for systemic and CNS lymphoma. Primary leptomeningeal lymphoma can present with symptoms of raised intracranial pressure or cranial or spinal polyradiculopathies. Diagnosis can be made by examining cerebrospinal fluid and incorporating immunophenotyping and molecular pathology techniques. Treatment options include irradiation and intrathecal or systemic chemotherapy. The features of PCNSL of T-cell origin and indolent B-cell PCNSL also are discussed.     

Treatment of primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) describes a malignant non-Hodgkin's lymphoma (NHL) whose sole site of involvement is the central nervous system (CNS). The diagnosis of PCNSL must be differentiated from systemic NHL with metastasis to the CNS, which usually occurs late in the course of systemic disease. PCNSL accounts for approximately 4% to 7% of primary brain tumors, and its incidence has been increasing since the mid-1970s. Compared with other more common malignant primary brain tumors, PCNSL tends to be more amenable to radiotherapeutic and chemotherapeutic intervention. In this article, the authors review the standard treatment for upfront and recurrent PCNSL.     

Case report of a patient with primary central nervous system lymphoma treated with radioimmunotherapy

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma arising within and confined to the central nervous system and, unlike other primary brain tumors, is very responsive to treatment. Aggressive management can lead to prolonged remissions or cures. However, the prognosis at relapse is generally poor with limited therapeutic options; clearly, new strategies are needed for these patients. Radioimmunotherapy has a growing role in the management of systemic non-Hodgkin lymphoma but has not been evaluated in PCNSL. We report here the first patient with PCNSL treated with radioimmunotherapy.     

Therapie von ZNS-Lymphomen

Primary central nervous system lymphoma (PCNSL) differs from nodal lymphoma with similar histological findings, usually diffuse large B-cell lymphoma, by its strong affinity for the central nervous system (CNS), aggressive course and unusual sensitivity to high-dose methotrexate (HDMTX). Thus, primary therapy of PCNSL is currently based on HDMTX but the optimal chemotherapy regimen has not yet been defined due to the rarity of this disease. In younger patients a cure should be the goal and thus intensified chemotherapy protocols should be considered. Whole brain irradiation does not prolong overall survival when used in primary therapy and thus should not be used routinely. Similar to PCNSL the infrequent CNS involvement of a systemic lymphoma, also called secondary CNS lymphoma, has a very poor prognosis. The scarce data suggest a role for HDMTX-based systemic chemotherapy and high-dose chemotherapy followed by stem cell transplantation for outcome improvement. Avoidance of late neurotoxicity is an important goal in the treatment of PCNSL.     

Advances in the Therapy of Primary Central Nervous System Lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin's lymphoma (NHL) confined to the nervous system. The management of PCNSL is quite different from the usual treatment of either primary brain tumors or systemic NHL. First-generation chemotherapy regimens used successfully in systemic NHL are ineffective in PCNSL, in large part due to the existence of the blood-brain barrier. Whole-brain radiation therapy (WBRT) results in high response rates but rapid relapse, and this treatment is associated with delayed neurotoxicity in patients with PCNSL. The addition of methotrexate-based chemotherapy has improved survival and lessened toxicity for this patient population. Fundamental issues that remain unresolved in PCNSL include identification of the optimal chemotherapy regimen for newly diagnosed and relapsed PCNSL, the role of WBRT and intrathecal chemotherapy in the treatment of PCNSL, and the optimal management of intraocular lymphoma. Finally, the optimal clinical study design for this rare disease has yet to be defined and implemented.     

Evaluation of Ac-Lys0(IRDye800CW)Tyr3-octreotate as a novel tracer for SSTR2-targeted molecular fluorescence guided surgery in meningioma

Journal of Neuro-Oncology - Primary central nervous system lymphoma (PCNSL) is a rare disease with a dismal prognosis compared to its systemic large B-cell lymphoma counterpart. Real world data are...     

Treatment of primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare neoplasm that has captured popular attention because of its rising incidence and marked chemosensitivity. It is a non-Hodgkins B-cell lymphoma (NHL) that appears confined to the central nervous system (CNS) at presentation but may be multifocal within the brain or involve the leptomeninges or eyes at diagnosis. Like systemic lymphoma, it is highly sensitive to corticosteroids, and administration of steroids should be withheld until the diagnosis has been confirmed histologically. Currently, the initial treatment of choice incorporates high-dose methotrexate (HD-MTX) either as a single agent or in combination with other systemic chemotherapies. Whole-brain radiotherapy (WBRT) can be a highly effective treatment modality when combined with MTX, but the combination causes an unacceptably high incidence of severe permanent neurotoxicity, particularly in patients over age 60. Therefore, chemotherapy alone is the initial treatment of choice in older patients. This approach is also being explored in younger patients, but it is possible that deferring radiotherapy may compromise disease control. Consequently, the role of radiotherapy remains to be clarified in newly diagnosed younger patients with PCNSL.     

原发性中枢神经系统淋巴瘤7例报道及文献复习

 本文报告原发性中枢神经系统淋巴瘤7例,病理类型均为B细胞型非何杰金氏淋巴瘤(NHL),全部病例采用手术和放射治疗,5例同时接受化疗,平均生存期26.1月,治疗失败主要原因是局部复发。作者结合文献对本病的发生、诊断和治疗进行讨论。     

Ocular involvement in primary central nervous system lymphoma: An increasing clinical problem?

A case of unilateral vitreous relapse in a patient with primary central nervous system lymphoma (PCNSL) following sequential systemic chemotherapy and radiotherapy is reported. A literature review reveals that ocular involvement may occur in up to 15–25% of cases during the course of this disease. Full ophthalmological assessment, including slit lamp examination, should be included in the routine staging of PCNSL at the time of initial diagnosis and subsequently if relapse occurs.     

甲氨蝶呤联合其他药物治疗原发中枢神经系统淋巴瘤研究进展

原发中枢神经系统淋巴瘤(PCNSL)是一种少见的结外淋巴瘤,近年发病率有增高趋势.化疗是PCNSL的最主要治疗手段,在各种化疗药物中,以大剂量甲氨蝶呤的疗效最为肯定.近年来国外对大剂量甲氨蝶呤联合其他药物的治疗方案开展了广泛的研究,文章就该方面的研究进展作一综述.     

Chemotherapy in newly diagnosed primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) accounts for only 3% of brain tumors. It can involve the brain parenchyma, leptomeninges, eyes and the spinal cord. Unlike systemic lymphoma, durable remissions remain uncommon. Although phase III trials in this rare disease are difficult to perform, many phase II trials have attempted to define standards of care. Treatment modalities for patients with newly diagnosed PCNSL include radiation and/or chemotherapy. While the role of radiation therapy for initial management of PCNSL is controversial, clinical trials will attempt to improve the therapeutic index of this modality. Routes of chemotherapy administration include intravenous, intraocular, intraventricular or intra-arterial. Multiple trials have outlined different methotrexate-based chemotherapy regimens and have used local techniques to improve drug delivery. A major challenge in the management of patients with PCNSL remains the delivery of aggressive treatment with preservation of neurocognitive function. Because PCNSL is rare, it is important to perform multicenter clinical trials and to incorporate detailed measurements of long-term toxicities. In this review we focus on different chemotherapeutic approaches for immunocompetent patients with newly diagnosed PCNSL and discuss the role of local drug delivery in addition to systemic therapy. We also address the neurocognitive toxicity of treatment.     

原发中枢神经系统恶性淋巴瘤的诊断及治疗

目的:探讨原发中枢神经系统恶性淋巴瘤(PCNSL)的临床、影像学表现,治疗方案选择和预后. 方法: 对24例PCNSL的诊断、治疗过程、预后进行回顾性分析. 结果: PCNSL的临床表现以颅内压增高、局灶占位性病变损伤症状为主,肿瘤可单发或多发;肿瘤影像学缺乏特异性,确诊依靠病理学诊断. 结论: PCNSL恶性度高,预后不良,手术全切率低;对确诊病例采用个体化的手术方案结合术后放、化疗是治疗本病的关键,可显著延长患者生存时间.     

Insights into the biology of primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare variant of non-Hodgkin lymphoma that is confined to the central nervous system. Biologic studies of PCNSL are challenging to conduct because the disease is rare and available tissue material is sparse. However, in recent years there has been progress in the understanding of PCNSL biology, largely as the result of multicenter studies using modern molecular techniques. Recent studies may improve insight into the pathogenesis of PCNSL and increase the chances of identifying prognostic factors and novel therapeutic targets. This review discusses recent advances in PCNSL biology, including immunologic and genetic risk factors, and focuses on the molecular alterations important in central nervous system lymphomagenesis.     

Primary central nervous system lymphoma: essential points in diagnosis and management

Primary central nervous system lymphoma (PCNSL) is an extra-nodal non-Hodgkin lymphoma. PCNSL is defined as lymphoma involving the brain, leptomeninges, eyes, or spinal cord without evidence of lymphoma outside the CNS. Treatment includes induction with chemotherapy and consolidation with whole-brain radiotherapy or high-dose chemotherapy supported by autologous stem cell transplantation. High-dose methotrexate is the most important drug in cases with PCNSL, and this drug will be used in combination with small molecules, BTK inhibitors, new monoclonal antibodies, and checkpoint blockers.     

Primary central nervous system lymphoma with testicular relapse

We report the case of a 54-year-old man with primary central nervous system lymphoma (PCNSL) who achieved a radiological complete response to high-dose methotrexate and 6 months later had a simultaneous local and testicular relapse.     

New treatment approaches in primary central nervous system lymphoma

A major advance in the treatment of primary central nervous system lymphoma (PCNSL) was made when the addition of chemotherapy to radiotherapy was shown to improve patient survival significantly. However, few chemotherapeutic agents are able to cross the blood-brain barrier. New therapeutic approaches in PCNSL are based on: the treatment of systemic non-Hodgkin's lymphomas with monoclonal antibodies and intensive chemotherapy followed by hematopoietic stem cell rescue, new chemotherapeutic agents with central nervous system penetration, or blood-brain barrier disruption to enhance drug delivery to the brain. This article discusses the rationale and results of these innovative approaches.     

原发性中枢神经系统淋巴瘤的诊断研究进展

 原发性中枢神经系统淋巴瘤（PCNSL）是一种临床上较为罕见的结外淋巴瘤,病理学分类多为弥漫性大B细胞淋巴瘤。WHO（2008）造血与淋巴组织肿瘤分类将"PCNSL"作为独立疾病实体列出。文章就近年PCNSL的临床诊断研究进展作一介绍。