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• We describe a patient presenting with persistent hiccups, who was found to have primary CNS lymphoma. • Persistent or intractable hiccups may reflect pathology within the brainstem, hypothalamic or supratentorial centres. • Appetite changes can be caused by hypothalamic tumour infiltration. • A first-order Horner's syndrome can arise from brainstem pathology. • A thorough neurological examination forms the cornerstone of localising neuropathology. We present a case of a 42-year-old male presenting with persistent hiccups and a Horner's syndrome, among other symptoms and signs of hypothalamic and brainstem dysfunction. He had a biopsy-proven diffuse infiltrative large primary CNS B-cell lymphoma involving the left fronto-temporal hemisphere, diencephalon and brainstem. The aim of this case report is to highlight key clinical and neuro-anatomical correlations that bring light to the art of the clinical examination. [ABSTRACT FROM AUTHOR]

• Radiological characteristics of central nervous system lymphoma. • Histological characteristics of central nervous system lymphoma. • Histological characteristics of lymphoma with steroid effect. • Administration of steroid prior to biopsy may affect diagnostic yield. Establishing the pathologic diagnosis of central nervous system (CNS) lymphoma can be challenging, yet management of this potentially curable disease depends heavily on it. One avoidable impediment to obtaining an accurate and timely diagnosis is the pre-operative administration of steroids, which causes tumor involution and prevents appropriate sampling of viable tissue. We discuss a case of primary CNS lymphoma that highlights the evolution of the disease and the attempts to establish a diagnosis in the setting of prior administration of corticosteroids. Familiarity with these clinical scenarios will help others avoid delays in patient care that results from delayed diagnosis. [ABSTRACT FROM AUTHOR]

Central nervous system lymphoma (CNSL) is a rare form of extranodal non-Hodgkin lymphoma. Central nervous system lymphoma can be primary (isolated to the central nervous space) or secondary in the setting of systemic disease. Treatment of CNSL has improved since the introduction of high-dose methotrexate and aggressive consolidation regimens. However, results after treatment are durable in only half of patients, and long-term survivors may experience late neurotoxicity, impacting quality of life. Given the rarity of this disease, few randomized prospective trials exist. This leaves many questions unanswered regarding optimal first-line and salvage treatments. Recent advances in the knowledge of pathophysiology of CNSL will hopefully help the development of future treatments. This review gives an overview of the epidemiology, pathophysiology, clinical presentation, diagnosis, and treatment of immunocompetent patients with CNSL. [ABSTRACT FROM AUTHOR]

• 18 patients with primary CNS lymphoma had received preoperative steroids. • Histopathological diagnosis was successfully made on these patients. • Short course of steroids may not obscure the tissue diagnosis of PCNSL. High dose corticosteroids are an effective tool for rapidly alleviating neurologic symptoms caused by intracranial mass lesions. However, there is concern that preoperative corticosteroids limit the ability to obtain a definitive pathologic diagnosis, particularly if imaging features suggest primary central nervous system lymphoma (PCNSL). To explore the impact of preoperative corticosteroids in newly diagnosed PCNSL patients, from 2009 to 2018 treated at our institution. We identified 54 patients; 18 had received corticosteroids prior to biopsy or resection. Only in one case did the patient have a prior non-diagnostic biopsy, requiring a second procedure. The cumulative doses of preoperative dexamethasone ranged from 4 mg to 120 mg (mean 32 mg, median 24 mg), given over 1–14 days (mean 2 days, median 1 day), and the majority had received corticosteroids for only 1–2 days. There was a trend for a larger diameter of lesional T1 contrast enhancement for patients who received steroids (39 mm vs. 34 mm, p = 0.11). In this series of cases with pathologically and clinically proven PCNSL, preoperative corticosteroids had been given in a third of cases, suggesting that they may be given for symptomatic relief without compromising pathologic diagnosis. Despite the commonly held tenet that preoperative corticosteroids can obscure the pathologic diagnosis in PCNSL, this is likely not the case in the majority of patients who receive a short course preoperatively. Obtaining a second stereotactic scan to confirm continued presence of the lesion prior to tissue sampling may also mitigate these concerns. [ABSTRACT FROM AUTHOR]

Abstract Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, which consists a subset of primary central system lymphoma (PCNSL) and the most common type of intraocular lymphoma. The involvement of eyes is estimated to be approximately 20% of PCNSL, but the brain involvement may be up to 80% of PVRL. Typically, PVRL is a high grade B-cell malignancy of the retina and needs to be assorted from choroidal low-grade B-cell lymphomas. Very often PVRL masquerades and can be erroneously diagnosed as chronic uveitis, white dot syndromes or other neoplasms. Establishing an accurate diagnosis may involve cytology/pathology, immunohistochemistry, flow cytometry, molecular pathology and cytokine profile analysis. There is inadequate information about PVRL's true incidence, ethnic/geographical variation and pathogenetic mechanisms. The therapeutic approach of PVRL involves aggressive chemotherapy and radiation therapy. Although PVRL tends to have a good response to the initial treatment, the prognosis is poor and the survival restricted due to the high relapse rates and CNS involvement. [ABSTRACT FROM AUTHOR]

Abstract Primary central nervous system lymphoma (PCNSL) is a rare and aggressive brain tumor. The prognosis is poor, with high rates of relapse and disease progression after treatment. In addition, PCNSL affects a largely older population, so that a significant proportion of patients are ineligible for intensive therapies and high-dose chemotherapy. The elderly patients are also susceptible to the accelerated and detrimental cognitive side effects of whole-brain irradiation which is an alternative consolidation to high-dose chemotherapy. Maintenance therapy has been shown to be a promising strategy to prolong remission time in other hematopoietic malignancies. Herein, we discuss the place of maintenance treatment in PCNSL in view of perspective obtained from hematological malignancies and non-Hodgkin's lymphoma. [ABSTRACT FROM AUTHOR]

• Sensorimotor deficits and ataxia were common amongst TDL. • Mild mass effect and lack of central enhancement are characteristics of TDL. • Peripheral diffusion restriction does not reliably differentiate TDL. • CNS lymphomas can closely mimic TDL. Differentiating tumefactive demyelinating lesions (TDL) from neoplasms of the central nervous system continues to be a diagnostic dilemma in many cases. Our study aimed to examine and contrast the clinical and radiological characteristics of TDL, high-grade gliomas (HGG) and primary CNS lymphoma (CNSL). This was a retrospective review of 66 patients (23 TDL, 31 HGG and 12 CNSL). Clinical and laboratory data were obtained. MRI brain at presentation were analyzed by two independent, blinded neuroradiologists. Patients with TDLs were younger and predominantly female. Sensorimotor deficits and ataxia were more common amongst TDL whereas headaches and altered mental status were associated with HGG and CNSL. Compared to HGG and CNSL, MRI characteristics supporting TDL included relatively smaller size, lack of or mild mass effect, incomplete peripheral rim enhancement, absence of central enhancement or restricted diffusion, lack of cortical involvement, and presence of remote white matter lesions on the index scan. Paradoxically, some TDLs may present atypically or radiologically mimic CNS lymphomas. Careful evaluation of clinical and radiological features helps in differentiating TDLs at first presentation from CNS neoplasms. [ABSTRACT FROM AUTHOR]

LETM is a common syndrome and the diagnosis of CNS lymphoma is not usually considered in the list of differentials. Primary CNS lymphoma can present as longitudinally extensive transverse myelopathy. Failure to suspect and evaluate leads to delay in diagnosis and treatment. PCNSL may be non contrast enhancing on gadolinium enhanced MRI. CSF analysis should be done preferably before starting corticosteroids as it is usual practice in treatment of transverse myelitis, as steroids may lead to transient improvement and mask the correct diagnosis. Repeated CSF examinations may be needed to clinch the diagnosis. [ABSTRACT FROM AUTHOR]

The article presents a case study on the association of toxoplasmosis gondii in focal retinochoditis and vitritis in large B-cell and primary intraocular lymphoma. A 79-year old woman was presented for treatment of persistent vitritis and recurrent ocular toxoplasmosis through vitrectomy. The study found that T. gondii caused the lymphoproliferation and development of primary intraocular lymphoma.

Summary: Natural killer (NK)/T-cell lymphomas represent a rare type of lymphoma derived from either activated NK cells or, rarely, cytotoxic T cells. These lesions are most commonly extranodal and tend to present as destructive lesions within the midline facial structures. Other than the nasal cavity and paranasal sinuses, several other extranodal sites of involvement have been reported, including the pharynx, gastrointestinal tract, and testis. Although secondary involvement of the central nervous system has been reported, a convincing case of primary brain NK/T-cell lymphoma has not been previously reported. Here, we report a case of primary brain lymphoma of NK/T-cell type with a characteristic phenotype expressing CD3ε, CD56, granzyme B, Epstein-Barr virus–encoded small nuclear RNAs, with germline T-cell receptor gene configuration, and showing an unusual intravascular component. The patient underwent extensive imaging studies, revealing only the brain lesion. The lymphoma failed to respond to therapy and the patient eventually died after transfer to a hospice facility. This unusual case highlights an unusual presentation of a rare disease entity and highlights the need for a better understanding of the biology and treatment of T-cell lymphomas. [Copyright &y& Elsevier]

This review article provides guidelines for the diagnosis, staging, and management of primary nervous system lymphoma based on the results of clinical trials conducted during the last decade. Recent progress in our understanding of the pathogenesis of primary nervous system lymphoma is summarized, and implications of these findings for the development of diagnostic tools and new therapeutic strategies are outlined. We performed a search of the PubMed database (National Center for Biotechnology Information) for articles on primary nervous system lymphoma published between 1970 and May 2005. Primary nervous system lymphoma affects the brain, eye, and meninges as well as cranial, spinal, and peripheral nerves. Although important lessons have been learned from the pathogenesis of extraneural non-Hodgkin's lymphoma, the unique organotropism of primary nervous system lymphoma remains poorly understood. Diagnosis is facilitated by modern imaging techniques and molecular markers. Clinically recognizable "precursors" may exist but frequently elude specific diagnosis. Insight into the peculiar pharmacokinetics of chemotherapy aimed at tumors within the nervous system has led to the development of methotrexate-based regimens that can achieve prolonged progression-free survival without the use of radiation. Long- term survival and, in selected cases, even a cure are possible in primary nervous system lymphoma. Treatment should be provided in specialized multidisciplinary centers. In spite of remarkable progress through methotrexate-based chemotherapy, the majority of patients experience relapse within a few years. Better diagnostic tools are required for earlier diagnosis and monitoring of treatment response. A deeper understanding of the pathogenesis of primary nervous system lymphoma may reveal new therapeutic targets. [ABSTRACT FROM AUTHOR]

• In situations of atypical MS, biopsy should be pursued early. • Lymphomatosis cerebri can mimic aggressive MS. • Lymphomatosis cerebri can be missed by biopsy as its CNS involvement is patchy. Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma with few cases reported. Here, we describe the case of a patient with clinical presentation, imaging, and biopsy in keeping with aggressive multiple sclerosis (MS) such as that in Marburg variant. He deteriorated clinically over 9 months. Post-mortem examination yielded a diagnosis of LC with B-cell lymphoma. LC is notoriously difficult to diagnose, as it can present in various ways and biopsy of unaffected areas will be non-diagnostic. In our case, diagnosis was made more challenging by the patient's dramatic response to treatment with steroids and cyclophosphamide. [ABSTRACT FROM AUTHOR]

Abstract: We report a now 74-year-old patient who was successfully treated with a methotrexate (MTX)-ssbased polychemotherapy protocol (Bonn protocol) for primary central nervous system lymphoma (PCNSL) in 1996. When presenting with an unusually late relapse after 13years of tumor-free survival the diagnosis was made on the basis of clinical and radiological criteria. In the context of the very limited treatment options for recurrent PCNSL, it is reassuring that the re-application of high dose-MTX-based polychemotherapy, including intraventricular treatment, again succeeded in a sustained complete response with still low neurotoxicity. [Copyright &y& Elsevier]

Abstract: Primary central nervous system lymphoma (PCNSL) is a rare tumor with a rising incidence in immunocompetent elderly and immunocompromised patients. The early diagnosis is important for quick initiation of appropriate treatment, although the clinical and radiological findings are frequently misleading. With the present case we show the usefulness of combining conventional magnetic resonance imaging (MRI) with magnetic resonance spectroscopy (1H MRS) for the confirmation of the diagnosis in an immunocompetent patient; in the absence of a “gold standard criteria for the diagnosis” both procedures are necessary and complementary investigations. [Copyright &y& Elsevier]