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BACKGROUND: Primary central nervous system lymphoma is rare, and primary central nervous system T cell lymphoma is relatively uncommon, contributing to < 5% of all cases. Lymphomatosis cerebri, a rare subtype of primary central nervous system lymphoma, is characterized by extensive white-matter lesions on magnetic resonance imaging and nonspecific symptoms, such as cognitive decline and depression. Reports of lymphomatosis cerebri in adult T cell leukemia/lymphoma are limited. CASE PRESENTATION: A 49-year-old Japanese man gradually developed insomnia, anorexia, and weight loss over a 2-month period following work-related promotion. Initially diagnosed with depression, his condition rapidly deteriorated with cognitive decline and motor dysfunction. Despite various treatments, his symptoms persisted within a month. Upon admission, the presence of neurological abnormalities suggestive of a central nervous system disorder raised suspicion of a cerebral lesion. Diagnostic tests revealed extensive brain lesions on imaging and the presence of atypical lymphocytes (flower cells) in the cerebrospinal fluid. The patient was diagnosed with lymphomatosis cerebri due to adult T cell leukemia/lymphoma, a rare presentation in the literature. Due to irreversible brainstem damage and poor neurological prognosis, aggressive treatment was not initiated, and the patient died, with an autopsy confirming the diagnosis. CONCLUSION: Lymphomatosis cerebri with adult T cell leukemia/lymphoma is very rare. It is crucial to promptly consider lymphomatosis cerebri as a differential diagnosis, particularly in cases of rapid cognitive decline and poor treatment response. Recognition of lymphomatosis cerebri as an important differential diagnosis for cognitive decline, and depression is necessary for timely intervention and management. Further research is required to better understand this unique and rare presentation in adult T cell leukemia/lymphoma.

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While NHL commonly affects lymph nodes, peritoneal lymphomatosis causing ascites is rare in pediatric patients. We present a unique case of DLBC lymphoma in a Peruvian child presenting as ascites associated with peritoneal lymphomatosis. The 11-year-old boy was admitted with ascites and dyspnea. Physical examination revealed collateral circulation, abdominal distension, and diminished breath sounds. Investigations led to a suspected diagnosis of peritoneal tuberculosis; however, a laparoscopic biopsy showed granulomatous infiltration consistent with high-grade diffuse B-cell lymphoma. The peritoneal lymphomatosis causing ascites is uncommon, and its initial presentation as peritoneal symptoms is even rarer. Differential diagnosis between peritoneal tuberculosis and DLBCL involvement can be challenging due to both shared signs and symptoms. Staging systems, such as the International Pediatric NHL Staging System, aid in determining the extent of the disease. DLBCL has a good prognosis, with treatment regimens such as the LMB-89 protocol showing high overall survival rates. Awareness of DLBCL's atypical presentations is crucial for timely diagnosis and management in the pediatric population. To conclude, children with ascites represent a diagnostic challenge posed by overlapping symptoms with other conditions, such as tuberculosis, and the need for a comprehensive approach to rule out different etiologies. Additionally, it is important the prompt treatment to avoid complications.

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Peritoneal lymphomatosis (PL) is a rare lymphoma-associated condition defined as the dissemination of lymphoma cells in the peritoneum. An 82-year-old man presented with abdominal pain, heartburn, and high fever. Radiological findings, including positron emission tomography-computed tomography (PET-CT), and gastrointestinal fiberscopy, showed diffuse thickening of the peritoneum, omentum, and mesentery; however, no lymphadenopathy, hepatosplenomegaly, or gastrointestinal lesions were observed. Under suspicion of peritonitis carcinomatosa of unknown origin, exploratory laparoscopy was performed that revealed multiple white nodules and masses on the surfaces of the peritoneum, mesentery, and intestinal serosa. The histopathological and cytogenetic findings of the peritoneum revealed high-grade B-cell lymphoma, not otherwise specified, and a gain of MYC by fluorescence in-situ hybridization. The patient was treated with two cycles of R-CHOP therapy, followed by six cycles of dose-adjusted EPOCH-R therapy, and a complete metabolic response was confirmed by PET-CT. Since there are no specific radiological findings to confirm the diagnosis of PL, a histopathological diagnosis is usually required. Most PL exhibit an aggressive lymphoma phenotype and can be cured by appropriate chemotherapy. Therefore, early diagnosis and treatment are desirable.

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Lymphomatosis cerebri (LC) is a rare type of primary central nervous system lymphoma with diffuse, nonenhancing infiltrative lesions and is often misdiagnosed. Our study aimed to investigate the clinical characteristics and prognosis of LC through analyzing patients from the literature and our own center, so as to improve early diagnosis and treatment. PubMed, Web of Science and our hospital databases were reviewed, and information on demographic, clinical, pathological, cerebrospinal fluid (CSF), neuroimaging and treatment options was extracted. Univariate survival analysis was conducted by generating survival curves and comparing them using the log-rank test. Multivariate analysis was performed using the Cox proportional hazards regression model to identify the prognostic predictors. A total of 81 patients (median age: 58 years; interquartile range, IQR: 50-66.5 years), 45 males and 36 females, were included. The most common symptoms were cognitive impairment (65.4%) and gait impairment (50.6%). Imaging studies indicated that all 81 patients had supratentorial structure involvement, and 93.8% (76/81) had bilateral hemisphere involvement. There were 53.3% (32/60) patients with CSF pleocytosis and 65% (39/60) patients with increased CSF protein levels. The median time of diagnosis was 4.8 months (IQR: 2.3-6.9 months). Compared with 4 (95% CI: 1.78-6.22) months for all 81 patients, the median OS was 20 (95% CI: 8.24-31.76) months for those who had chemotherapy plus radiotherapy. Multivariate Cox analysis revealed that chemoradiotherapy (HR: 0.12; 95% CI: 0.02-0.68) and higher CSF glucose level (HR: 0.01; 95% CI: 0.00-0.26) were inversely associated with death. The diagnosis of LC should be alerted when neuroimaging with bilateral hemispheric involvement and CSF abnormality with pleocytosis and increased protein. Once the diagnosis is confirmed, the combination of chemotherapy and radiotherapy can be considered if the patient's physical condition permits.Journal standard instruction requires an unstructured abstract. Kindly check and confirm.We have checked and confirmed that there is no problem.

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Leukoencephalopathy is a common finding on Magnetic Resonance Imaging (MRI), particularly in the elderly. A differential diagnosis may represent a very bet for clinicians when clear elements for diagnosis are lacking. Diffuse infiltrative "non mass like" leukoencephalopathy on MRI may represent the presentation of a very rare aggressive condition known as lymphomatosis cerebri (LC). The lack of orienting data, such as contrast enhancement on MRI or specific findings on examination of Cerebrospinal Fluid (CSF) or blood tests, may even far more complicate such a difficult diagnosis and orientate toward a less aggressive but time-losing mimic. A 69-old man initially presented to the Emergency Department (ED) complaining the recent appearance of unsteady walking, limitation of down and upgaze palsy, and hypophonia. Brain MRI revealed the presence of multiple, confluent hyperintense lesions on T2/Flair Attenuated Imaging Recovery (FLAIR) sequences involving either the withe matter of the semi-oval centres, juxtacortical structures, basal ganglia, or bilateral dentate nuclei. DWI sequences showed a wide restriction signal in the same brain regions but without any sign of contrast enhancement. Initial 18F-labeled fluoro-2-deoxyglucose positron emission tomography (FDG PET) and CSF studies were not relevant. Brain MRI revealed a high choline-signal, abnormal Choline/ N-Acetyl-Aspartate (NAA), and Choline/Creatine (Cr) ratios, as well as reduced NAA levels. Finally, a brain biopsy revealed the presence of diffuse large B-cell lymphomatosis cerebri. The diagnosis of lymphomatosis cerebri remains elusive. The valorisation of brain imaging may induce clinicians to suspect such a difficult diagnosis and go through the diagnostic algorithm.

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Diagnosis of lymphomatosis cerebri (LC) is usually delayed because of its rarity and the need for pathological confirmation. The association of LC with humoral immunity has scarcely been reported. Herein, we present a woman with a 2-week history of dizziness and gait ataxia, followed by diplopia, altered mental status, and spasticity of all limbs. Magnetic resonance imaging (MRI) of the brain showed multifocal lesions involving bilateral subcortical white matter, deep gray structures, and brainstem. Oligoclonal bands and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies were present in cerebrospinal fluid (CSF) twice. She was initially treated with methylprednisolone but still worsening. A stereotactic brain biopsy confirmed the diagnosis of LC. This is a report on the distinctive coexistence of the rare CNS lymphoma variant and the anti-NMDAR antibody.

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Intravascular lymphoma (IVL) is characterized by the proliferation of large malignant lymphocytes within the lumen of blood vessels. This retrospective, multi-center, case series study aimed to describe the MRI features of confirmed central nervous system IVL in dogs and compare them with histopathological findings. Medical record databases from seven veterinary centers were searched for cases of histologically confirmed IVL. Dogs were included if an MRI was performed. The MRI studies and histopathology samples were reviewed to compare the MRI changes with the histopathological findings. Twelve dogs met the inclusion criteria (12 brains and three spinal cords). Imaging of the brains revealed multifocal T2-weighted/FLAIR hyperintense and T1-weighted iso-hypointense lesions, with variable contrast enhancement; areas of abnormal diffusion both in arterial and venous territories in diffusion-weighted imaging; and meningeal enhancement. On gradient echo images (GRE), the changes comprised tubular susceptibility artifacts, consistent with the "susceptibility vessel sign", and additional variably sized/shaped intraparenchymal susceptibility artifacts. Spinal cord lesions presented as fusiform T2-weighted hyperintensities with scattered susceptibility artifacts on GRE and variable parenchymal and meningeal contrast enhancement. On histopathology, subarachnoid hemorrhages and neuroparenchymal areas of edema and necrosis, with or without hemorrhage, indicating ischemic and hemorrhagic infarctions, were found. These lesions were concurrent with severely dilated meningeal and parenchymal arteries and veins plugged by neoplastic lymphocytes and fibrin. Due to the unique angiocentric distribution of IVL, ischemic and hemorrhagic infarcts of variable chronicity affecting both the arterial and venous territories associated with thrombi formation can be detected on MRI.

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Two equine patients presented separately with severe abdominal distention, colic, lethargy, and decreased appetite. An ante-mortem diagnosis of lymphoma was reached in each case based on peritoneal fluid cytology. Due to a poor prognosis, the horses were humanely euthanized. Post-mortem examination with histology and immunohistochemistry confirmed both cases as lymphoma: alimentary B-cell lymphoma of the distal jejunum and cecum in one case, and T-cell lymphoma of the cecum in the second case. Both cases exhibited extensive metastasis with peritoneal and pleural serosae covered in small nodules and plaque like masses consistent with lymphomatosis. These cases document a unique presentation of lymphoma in equine patients presenting as peritoneal lymphomatosis with ascites.

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INTRODUCTION: Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma that diffusely involves throughout the brain. In recent years, increasingly reported cases have notably broadened the spectrum of clinical and radiological features; however, it remains a great diagnostic challenge. CASE REPORT: We reported an atypical case of LC presented with subacute onset of focal neurological deficits and diffuse T2 hyperintensities without contrast enhancement on magnetic resonance imaging. He was initially considered as inflammatory leukoencephalopathy and received empirical corticosteroids, showing a dramatically clinical response. Three months later, the patient relapsed with deteriorating symptoms and enlarged brain lesions with mass-like enhancement. A diagnosis of LC was finally established according to the radiological and pathological findings. DISCUSSION: Though rare, LC should always be kept as a differential diagnosis of diffuse leukoencephalopathy. Neurologists should be aware of every detailed information about LC to avoid a delay of diagnostic biopsy in clinical practice.

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ABSTRACT Introduction: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. Description of the case: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. Discussion: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. Conclusion: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.

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Lymphomatosis cerebri is an atypical form of primary central nervous system lymphoma (PCNSL), which frequently causes rapid progression of dementia. A 68-year-old woman exhibited rapidly progressing disorientation and a mini-mental state examination score of 9. The fluid-attenuated inversion recovery of a magnetic resonance image (MRI) demonstrated focal areas of high-signal intensity in the right frontal lobe with a small enhancement, which was histologically diagnosed as diffuse large B-cell type lymphoma. The lesion dramatically shrank, and no enhancements were identified on MRI after treatment with high-dose methotrexate (MTX) and whole-brain radiation (WBR). However, her recovery of cognitive function was poor. The patient visited our clinic every 2 months but succumbed to systemic mycotic sepsis 14 months after the biopsy. Autopsy revealed lymphomatosis cerebri in the patient based on a feature of scattered small clusters of lymphoma cells infiltrating into the brain parenchyma in both cerebral hemispheres. Differentiation of lymphomatosis cerebri from other white matter degenerative diseases is usually challenging because lymphomatosis cerebri seldom forms mass lesions. In lymphomatosis cerebri, the lymphoma cells infiltrate into several regions in the brain tissue, including the basal ganglia, brainstem, and corpus callosum, in addition to periventricular and subcortical white matters. The rapid deterioration of cognitive function in the patient suggests a rapid spread of lymphomatosis cerebri, necessitating early histological diagnosis and prompt treatments. If the diagnosis is obtained, administration of high-dose MTX and WBR followed by rituximab and cytarabine can contribute to a longer survival time, based on our literature review.

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Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma (PCNSL). It is characterized by diffuse infiltration of atypical lymphoid cells with no mass formation and little or no contrast enhancement on magnetic resonance imaging (MRI). Interestingly, some lymphoma cells form characteristic spindle shapes; these cells are found in some variants of malignant lymphoma, such as primary cutaneous follicle center lymphoma, but they have not been reported in PCNSL or LC. Here, we provide an autopsy case report of LC in a 69-year-old immunocompetent man who developed rapidly progressive cognitive decline and died on day 68 after the episode despite treatment with intravenous methylprednisolone administration. MRI revealed high signal intensities on T2-weighted images of the cerebral hemispheres, cerebellum, brainstem, and spinal cord without gadolinium enhancement on T1-weighted images. On autopsy, diffuse infiltrative atypical cells were seen; these cells were positive for CD20 and CD79a and negative for GFAP, CD3, and CD5 on immunohistochemistry, resulting in a diagnosis of diffuse large B-cell lymphoma, specifically LC. We found characteristic spindle-shaped cells, especially in the cerebral cortex. This is the first report showing that lymphoma cells in PCNSL can take on a spindle-shaped form. It is difficult to recognize these spindle-shaped cells as lymphoma cells on hematoxylin and eosin staining and diagnose them correctly with small biopsy specimens without immunohistochemistry. This case suggests that we should add atypical, spindle-shaped cells to the differential diagnosis of PCNSL.

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Peritoneal lymphomatosis (PL) is a rare presentation of malignant lymphoma cases, many of which are diagnosed as diffuse large B-cell lymphoma (DLBCL) and characterized by aggressive clinical courses. We herein report a 63-year-old woman presenting with the rapid development of abdominal distention due to bulky peritoneal tumors. The pathological evaluation of a needle biopsy sample, combined with flow cytometry, yielded the diagnosis of DLBCL. Prompt chemotherapeutic intervention resulted in favorable disease control and sustained complete remission. It is necessary to diagnose cases of DLBCL presenting as PL early to ensure prompt treatment and prevent mortality.

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INTRODUCTION: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. DESCRIPTION OF THE CASE: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. DISCUSSION: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. CONCLUSION: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.

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Peritoneal lymphomatosis is extremely rare and associated with poor prognosis. Most practitioners only pay more attention to peritoneal carcinomatosis. However, peritoneal lymphomatosis can be neglected and misdiagnosed. We report a teenager with 10 days of abdominal distension and pain accompanied by computed tomography scan suggesting diffuse thickening of the peritoneum and omentum and abdominopelvic effusion. Tuberculous peritonitis and peritoneal carcinomatosis were initially suspected. However, it was finally confirmed as non-Hodgkin's B-cell lymphoma by omentum biopsies. He achieved complete remission after chemotherapy and autologous stem cell transplantation. But unfortunately, he suffered a relapse and died 10 months after diagnosis. Following a review of the literature, it can be concluded that the discovery of lymphomatosis in peritoneum is a rare finding. Lymphoma should be considered in the differential diagnosis of unexplained peritoneal thickening on computed tomography, and this case emphasizes the importance of early pathological diagnosis to make sure that the right treatment can be started opportunely.

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Lymphomatosis cerebri (LC) is a significant challenge in terms of its clinical diagnosis due to it being a rare disease. The purpose of the present study was to investigate the multimodality imaging characteristics, clinical features and reasons for misdiagnosis of LC with the goal of potentially facilitating early and accurate diagnosis of this frequently misdiagnosed disease. In the present study, clinical data and cerebral multimodality imaging findings from 11 patients with LC proven based on pathology were retrospectively analyzed and reviewed with consultation of the literature. The results indicated that the common symptoms included cognitive decline (8/11), gait disturbance (9/11) and behavioral abnormalities (5/11). Cerebrospinal fluid analysis indicated that the number of cells and level of protein increased (8/10). All patients had both deep and lobar lesion distribution of bilateral cerebral white matter with equal or slightly low-density shadows on CT plain scan and slightly longer signals on T1- and T2-weighted MRI. Most of the lesions (9/11) exhibited isointensity or slight hyperintensity on diffusion-weighted imaging and hyperintensity on apparent diffusion coefficient maps. In addition, five patients presented with a marked decrease in N-acetylaspartate/creatine (Cr) and increase in choline/Cr on 1H-magnetic resonance spectroscopy, including an increase in lipid/Cr in 3 cases. Of these, one case exhibited no increase in lesion metabolism and 2 cases had slightly increased uptake on positron emission tomography/CT. The present study indicated that the multimodality imaging findings of LC have certain distinct characteristics and prompt recognition of these features may significantly improve early diagnosis and patient prognosis. Misdiagnosis may be mainly due to insufficient understanding knowledge of the condition and improper brain biopsy.

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Hashimoto's encephalopathy (HE) is an autoimmune encephalopathy that presents with various clinical symptoms, including cognitive deterioration, convulsive seizures, and personality changes. HE is associated with thyroid autoimmunity; however, few cases have been reported to develop as paraneoplastic syndrome. Herein, we report the case of a 73-year-old woman with onset of rapidly progressive dementia. Brain magnetic resonance imaging showed diffuse T2 hyperintensity areas involving the bilateral cerebral white matter, right midbrain tegmental area, left cerebral peduncle, and right middle cerebellar peduncle without clear diffusion hyperintensities and gadolinium enhancement. Her neurological symptoms worsened rapidly, and she presented with the apallic syndrome. Electroencephalogram showed periodic synchronous discharge, suggestive of Creutzfeldt-Jakob disease. However, a brain biopsy revealed infiltration of atypical lymphoid cells expressing CD20, and the anti-NH2 terminal of the α-enolase antibody was detected, diagnosing the complication with lymphomatosis cerebri and HE. High-dose intravenous methylprednisolone therapy and oral prednisolone with whole cranial irradiation enabled her to have simple conversations and consume food orally; however, severe cognitive impairment persisted. Although HE is a rare complication of malignant lymphoma, clinicians should be aware that it could be strongly suspected if the clinical symptoms worsen in the absence of imaging changes.

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OBJECTIVES: Diffuse infiltrative "non-mass-like" parenchymal lesions on MRI brain are a known presentation of an aggressive condition called lymphomatosis cerebri (LC) but are often misdiagnosed due to its non-specific clinical and imaging findings. We aim to identify clues to differentiate lymphomatosis from its less aggressive mimics based on imaging features. MATERIAL AND METHODS: MRI brain studies showing diffuse infiltrative "non-mass-like" parenchymal lesions between January 2013 and March 2020 were retrospectively identified and read for lesion location, signal characteristics, and enhancement pattern by two radiologists. Additional findings on MRI spine and whole-body fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) were recorded wherever available. The clinical diagnosis, patient demographics, symptoms, laboratory and histopathology results, treatment details, and follow-up details were also noted. RESULTS: Of the 67 patients, 28 (41.7%) were diagnosed with lymphomatosis. The remaining 39 (13.4%) patients were classified as non-lymphomas (infective, vasculitis, and inflammatory conditions). Diffusion restriction on MRI (20/67, P = 0.007) and increased regional activity on FDG PET-CT (12/31, P = 0.017) were the two imaging parameters found to significantly favor lymphomatosis over other conditions, whereas the presence of microhemorrhages on susceptibility-weighted imaging was significantly associated with vasculitis (P = 0.002). Rapid clinical or imaging deterioration on a short trial of steroids (P = 0.00) was the only relevant clinical factor to raise an early alarm of lymphomatosis. Positive serological markers and non-central nervous system systemic diseases were associated with non-lymphomatous diseases. CONCLUSION: LC and its less aggressive mimics can be differentiated on diffusion-weighted imaging-MRI and PET-CT when read in conjunction with rapid progression of clinical features, serological workup, and systemic evaluation.