Primary leptomeningeal lymphoma

“…This result is not specific for leukaemic involvement but may be related to the enhancement of regulatory T cells previously described in lymphomatous and carcinomatous meningitis (Haas et al, 2008). Sensitivity of MRI is limited in the detection of leptomeningeal disease (K€ uker et al, 2005;Kiewe et al, 2010;Pauls et al, 2012;Taylor et al, 2013). However, if CNSi with CLL is suspected, neuroimaging may be a valuable diagnostic tool.…”

Section: Discussionmentioning

confidence: 96%

Management of central nervous system involvement in chronic lymphocytic leukaemia: a retrospective cohort of 30 patients

et al. 2016

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SummaryCentral nervous system involvement (CNSi) is a rare and poorly reported complication of chronic lymphocytic leukaemia (CLL). Establishing cause and effect between the CLL and the neurological symptoms remains challenging. We have analysed a retrospective cohort of 30 CLL patients with CNSi, documented by lymphocytic infiltration either by flow cytometry of the cerebrospinal fluid (CSF; n = 29) or CNS biopsy (n = 1). Neurological symptoms were heterogeneous. At the time of CNSi, less than half of the patients had a progressive CLL and 20 had never been treated for CLL. Initial treatment with fludarabine-based immuno-chemotherapy, with or without intra-CSF therapy, led to durable response in eight out of nine untreated patients. In contrast, 50% patients receiving various prior treatments needed additional therapy within a median of 4 months (1-16). Ibrutinib led to complete response in 4/4 heavily pre-treated patients. From CNSi, 5-year overall survival was 72% and 48% for treatment-na€ ıve and previously treated patients respectively (P = 0Á06); 5-year progression-free survival (PFS) was 43% and 0% (P = 0Á125). 17p deletion was significantly associated with poor PFS (P = 0Á006). CNSi may be the only sign of progression of CLL and should be considered an initiation criterion of systemic treatment. Prognosis seemed to be related to CLL characteristics rather than to CNSi itself.

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“…Should systemic lymphoma be revealed in advance, invasion of the CNS may be included in the differential diagnosis, regardless of how distorted the CT or MRI findings are. Although primary leptomeningeal or dural lymphoma is rare (7,8), secondary CNS invasion is not. CNS involvement in non-Hodgkin's lymphoma tends to occur early, at a median of 5-6 months subsequent to the primary diagnosis of systemic lymphoma (3).…”

Section: Discussionmentioning

confidence: 99%

Secondary central nervous system lymphoma surrounding a region injured by subarachnoid hemorrhage and subsequent aneurysmal clipping

Murata

Hata²,

Noda³

et al. 2017

Biomedical Reports

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Abstract. The present case study describes a rare case of secondary central nervous system (CNS) lymphoma that infiltrated the dura and leptomeninges around the area injured by subarachnoid hemorrhage and subsequent aneurysmal clipping. Invasion of the CNS was observed by computed tomography as slurred fissures of the right parietal lobe adjacent to the surgery area. Subdural and subarachnoid enhancement overlapping the area injured by past surgical procedures was observed by contrast-enhanced magnetic resonance imaging. Surgical resection revealed B-cell lymphoma infiltrating the dura and leptomeninges surrounding the post-hemorrhagic area. The patient was subsequently diagnosed with systemic lymphoma and bone marrow invasion, and multiple lymph node swelling. To the best of our knowledge, this is the first report of malignant lymphoma involving the CNS overlapping a previously injured area. IntroductionLeptomeningeal infiltration is frequently encountered (1), and dural infiltration is not rare with secondary central nervous system (CNS) lymphoma (1,2). However, unless systemic lymphoma has already been identified, a finding of subdural and subarachnoid abnormality would not be directly linked to lymphoma (3). Furthermore, computed tomography (CT) and magnetic resonance imaging (MRI) abnormalities surrounding hemorrhagic and postoperative changes make it difficult to distinguish between onset of disease and postoperative complications, including metal artifacts. However, malignant cells that enter the CNS appear first in the dura and subarachnoid space in rats with a blood brain barrier (BBB) disrupted by focal injury following exposure to a cold temperature (4). Previous history of subarachnoid hemorrhage (SAH) and aneurysm clipping may be associated with CNS infiltration in systemic lymphoma. The current study describes a particularly rare case of systemic lymphoma involving the CNS overlapping with the area of injury to the dura and leptomeninges due to an aneurysmal SAH. To the best of our knowledge, no similar cases have previously been reported. Case reportA 56-year-old woman with a history of aneurysm clipping following acute SAH due to rupture of the right middle cerebral arterial aneurysm 6 years earlier (Fig. 1) was hospitalized at Kochi Health Sciences Center for sudden numbness of the left arm. The patient had not experienced malignant or benign diseases within the 6 years since discharge from hospital. The patient had been asymptomatic except for partial paralysis due to SAH. Unenhanced CT was performed to exclude the recurrence of SAH, and it revealed slurred fissures of the right parietal region and enlargement of the low-density area surrounding the preceding hemorrhagic scar was suspected (Fig. 2). In addition, MRI was performed (Fig. 3). Diffusion-weighted imaging (DWI) demonstrated a thin crescent of hyperintensity in the right temporo-occipital region, although the parenchyma near the right Sylvian fissure exhibited loss of signal with distortion due to a magnetic susceptibility artifact. F...

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Primary leptomeningeal lymphoma

Cited by 74 publications

References 33 publications

Ibrutinib responsive central nervous system involvement in chronic lymphocytic leukemia

Ibrutinib responsive central nervous system involvement in chronic lymphocytic leukemia

Management of central nervous system involvement in chronic lymphocytic leukaemia: a retrospective cohort of 30 patients

Secondary central nervous system lymphoma surrounding a region injured by subarachnoid hemorrhage and subsequent aneurysmal clipping

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