Intracranial Lymphoma Research Articles

RARE-03PRIMARY DURAL LYMPHOMA: SINGLE INSTITUTION STUDY OF TREATMENT AND OUTCOME

BACKGROUND: Primary dural lymphoma (PDL) is a rare form of primary intracranial lymphoma. Clinical and radiographic presentation often suggest a meningioma. Pathology often shows a low grade B-cell marginal zone lymphoma (MZL). MZL is very radiosensitive to relatively low dose of RT. Previous series have reported a high risk for systemic recurrence. METHODS: A retrospective review of primary dural lymphoma treated at the University of Miami Heath System between 08/2004-10/2014. RESULTS: Eleven patients were identified. Six were women. Median age at diagnosis was 48 years (range: 30 to 68). The most frequent symptoms at presentation were seizure (40%), headache (40%) and cranial nerve palsies (37%). Brain-MRI showed a single extra-axial enhancing lesion in 9 patients and multiple in two. Work-up was negative for systemic and leptomeningeal disease in all patients. Two patients had a history of HIV at diagnosis. All patients underwent resection/biopsy with an initial diagnosis of meningioma. Pathology showed marginal zone B-cell lymphoma (MZL) in all patients. One patient refused RT and was treated with rituximab. Ten patients were treated with focal RT. One of these patients refused to continue RT after been treated with 16 Gy. All others received between 30.6 to 36 Gy. Nine patients achieved complete response (CR) and two were lost to follow-up. Median follow-up was 3.3 years (range, 0.7 to 10 years). No patient developed a systemic relapse. Two patients developed CNS recurrences. One of these patients had progression of disease after being treated with 16 Gy and had a second course of RT (36 Gy), achieving CR. Another patient was treated with bendamustine/rituximab at progression and achieved CR. All patients are alive. CONCLUSIONS: Patients with PDL have a good prognosis. CR can be achieved with relatively low dose focal RT. No systemic relapses were observed in our series.

Uveitis ‐ the classic masquerade in adults

SummaryThe classic masquerade syndrome in adults is vitreoretinal lymphoma mimicking bilateral uveitis – in about one third of cases, before any evidence of an intracranial lymphoma. It is, however, not the sole neoplastic disease that can lure the clinician to diagnose uveitis. Other examples include posttransplant lymphoproliferative disorder, multiple myeloma, leukemia, histiocytosis, and carcinoma or another amelanotic tumour metastatic to the anterior segment of the eye, and certain paraneoplastic disorders. Making the correct diagnosis needs prior knowledge, a high index of suspicion, careful systemic workup and, quite often, a confirmatory fine needle aspiration biopsy. A further caveat is that in some of these disorders, immunosuppressive treatment may initially appear to be effective. Notably, this apparent effect will prove only temporary, whereas posttransplant lymphoproliferative disorder actually calls for less intensive immunosuppression. This talk will introduce these principles using clinical examples.

Primary Intracranial Mucosa-Associated Lymphoid Tissue Lymphoma

Low-grade B cell non-Hodgkin lymphomas typically arise from the marginal zone of the secondary lymphatic follicles. Their intracranial expression is very rare, most frequently affecting the dura mater and the choroid plexus glomi in the lateral ventricles. Their initial evaluation requires the exclusion of more common extra-axial lesions, such as meningiomas, dural metastasis, granulomatous lesions or secondary lymphoproliferative dural extension from body lymphomas. Whenever a ventricular lesion is present, the patient's age and lesion location help narrow the differential diagnosis. Dural-based lymphomas and ventricular/choroid plexus lymphomas are slow-growing lesions with imaging features similar to meningiomas, which is typically their main differential consideration. Diffusion-weighted images frequently show restricted diffusion behaviour on lymphomas, helping to differentiate them from the typical meningiomas.

MR imaging features of intracranial primary CNS lymphoma in immune competent patients.

We aimed to characterize specific MRI findings seen in immune competent patients with intracranial primary CNS lymphoma (PCNSL) and to determine their value in the management of such patients. Pre-treatment MRI examinations of 21 immunocompetent patients with biopsy-proven PCNSL were retrospectively evaluated. T1 and T2 signal characteristics as well as contrast enhancement features are described in all patients. Diffusion, perfusion and proton-MR-spectroscopy features are described in a subset of these patients. In the proper clinical and radiologic setting, suggesting the diagnosis of PCNSL can help institute proper treatment in a timely fashion and avoid unnecessary attempts at surgical resection and the associated morbidity.

Classical Hodgkin Lymphoma involving the central nervous system (brain) - an unusual presentation.

Key Clinical MessageIntracranial Hodgkin lymphoma (HL) is considered so atypical that an intracranial space-occupying lesion in a patient with known HL should be thoroughly investigated to rule out a second disease process.

Skull Base Lymphoma: Radiographic Presentation and Surgical Management

Introduction: The frequency of lymphoma involving the central nervous system has been increasing over the last few decades, even among immunocompetent patients. Radiographically, lymphoma has been known to mimic different disease processes. Therefore, surgeons of the head and neck usually include this disease when formulating a differential diagnosis. We describe multiple cases of intracranial lymphoma that were initially consistent with other types of tumors and their surgical management as done via an endoscopic endonasal approach (EEA). Case Reports: Case #1: 69-year-old female presented with a several month history of headache and an associated 2 week history of double vision. An MRI of her brain showed a clival tumor with associated mass effect onto the cavernous sinuses bilaterally; the lesion was consistent with a chordoma. Via an EEA, a successful debulking of her cavernous sinus was achieved after her frozen section result showed evidence of lymphoma. Her final pathology was found to be diffuse large B cell lymphoma. Case #2: 57-year-old male presented with a history of headache and diplopia. A brain MRI was ordered that showed a heterogeneously enhancing clivus that appeared abnormally expanded. There was also extraosseous enhancement into the cavernous sinuses and superior orbital fissures bilaterally, the sella, as well as Meckel's cave on the left. He proceeded with an EEA and his superior orbital fissures were decompressed successfully. Final pathology was consistent with diffuse large B cell lymphoma. Case #3: 76-year-old male presented with a history of recent epistaxis. An MRI of his orbits was obtained which showed a mostly homogenously enhancing mass involving the left nasal cavity and bilateral ethmoid air cells. There was extension of this mass into the left sphenoid sinus and left orbit with associated enhancement of the skull base involving the cribiform plate and planum sphenoidale. He presented for an EEA to resect the mass. Frozen section was consistent with esthesioneuroblastoma. However, final pathology proved to be large B cell lymphoma. Case #4: 58-year-old male presented with a one-month history of deteriorating left eye function. He had an MRI of his brain completed which showed a large, heterogeneously enhancing lesion of his sella with invasion into the bilateral cavernous sinuses, greater on the left than the right. The most likely diagnosis given to the lesion based on imaging was a pituitary macroadenoma. He had an EEA done and frozen section corroborated this diagnosis. During the procedure, the left cavernous sinus and the superior orbital fissures were decompressed. Final histologic analysis revealed diffuse B cell lymphoma. Conclusion: Lymphoma should always be included in the differential when considering tumors that invade and expand normal osseous components and dural compartments of the intracranial space. Because patients with expansile skull base lesions typically have cranial nerve involvement, decompressing foramen or bone passages through which these nerve pass is an important goal during surgery to regain function. In our opinion, often biopsy alone is not sufficient to manage these patients' disease process.

Primary Central Nervous System Anaplastic Large Cell Lymphoma is an Unusual Brain Tumour

We present a case of a 64-year-old man who was diagnosed with a primary anaplastic large cell lymphoma of the central nervous system (PCNSAL). He had received radical chemotherapy and radiotherapy for a non-small cell lung cancer (NSCLC) in the past. There is no known association between NSCLC and PCNSAL. We describe the diagnostic and therapeutic challenges associated with these rare intracranial lymphomas and highlight the potential role of newer biological agents in patients with anaplastic lymphoma kinase (ALK-1) positive PCNSAL.

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English

Primary CNS lymphoplasmacytic lymphoma: A case report and review of literature

Lymphoplasmacytic lymphoma is a chronic lymphoproliferative disorder characterized by a proliferation of plasma cells, small lymphocytes, plasmacytoid lymphocytes and the production of monoclonal IgM. Primary central nervous system lymphomas (PCNSL) are rare non-Hodgkin lymphomas (NHL) that can be found in the brain, leptomeninges, eyes or spinal cord, and are mostly intracerebral. PCNSLs constitute 3-4% of primary brain tumors, and in most cases are diffuse large B-cell lymphomas (DLBCL).(1) Low grade lymphomas as primary central nervous system (CNS) lymphoma are very rare. We present here a case report of a woman who presented with headache and was found to have primary intracranial lymphoplasmacytic lymphoma (LPL).

Differentiation of Newly Diagnosed Glioblastoma Multiforme and Intracranial Diffuse Large B-cell Lymphoma Using 11C-Methionine and 18F-FDG PET

The aim of our study was to examine the usefulness of PET with C-methionine (MET) and F-fluorodeoxyglucose (FDG) in the differentiation of glioblastoma multiforme (GBM) and intracranial diffuse large B-cell lymphoma (DLBCL). We evaluated 22 patients retrospectively with an enhancing brain tumor on MRI, including 15 GBM and 7 DLBCL, which was confirmed by histopathology. Dynamic PET scans with MET and FDG were performed for preoperative differential diagnosis. We assessed the images qualitatively and quantitatively. In quantitative assessment, the SUVmax was used on FDG PET and both late and early phases on MET PET. In addition, the ratio of SUVmax in the late and early phases on MET-PET was evaluated (ΔSUVmax). SUVmax on FDG PET of DLBCL was significantly higher than that of GBM. Setting an SUVmax of 12.0 as the cutoff for differentiating DLBCL from GBM, 1 GBM and 1 DLBCL were found to be false-positive and false-negative, respectively.SUVmax in the late and early phases of MET-PET was not significantly different between DLBCL and GBM; however, we also found significant differences in ΔSUVmax on MET-PET. Using ΔSUVmax 1.17 as the cutoff, we could differentiate DLBCL from GBM completely. In the present study, ΔSUVmax on MET-PET was slightly superior to SUVmax on FDG PET. Both SUVmax on FDG PET and ΔSUVmax on MET-PET were considered to be good diagnostic tests when encountering difficulties in this differential diagnosis.

CONCURRENT MEDICAL CONDITIONS AND LONG‐TERM OUTCOME IN DOGS WITH NONTRAUMATIC INTRACRANIAL HEMORRHAGE

Nontraumatic intracranial hemorrhage is bleeding originating from the brain or surrounding structures. It results from blood vessel rupture and may be primary or secondary in origin. The magnetic resonance imaging (MRI) characteristics of 75 dogs with nontraumatic intracranial hemorrhage were reviewed to determine signalment; intracranial compartment involved, size and number of lesions; type and prevalence of concurrent medical conditions; and long-term outcome. Hemorrhagic lesions were intraparenchymal (n = 72), subdural (n = 2) or intraventricular (n = 1). Thirty-three of 75 dogs had a concurrent medical condition. A concurrent condition was detected in 13 of 43 dogs with a single lesion ≥5 mm and included Angiostrongylus vasorum infection, intracranial lymphoma and meningioma. Of the 20 dogs with multiple lesions ≥5 mm, 7 had A. vasorum infection, 2 had hemangiosarcoma metastasis, 5 had suspected brain metastasis, and 1 was septicemic. Of the 12 dogs with multiple lesions, 2 had hyperadrenocorticism, 2 had chronic kidney disease, and 1 had hypothyroidism. Of these five dogs, all were hypertensive and four died within 12 months. No dog had a single lesion <5 mm. Long-term outcome was favorable in 26 of 43 dogs with single lesions ≥5 mm, 6 of 20 dogs with multiple lesions ≥5 mm, and 8 of 12 dogs with multiple lesions <5 mm. A. vasorum infection was the most common concurrent condition in dogs with nontraumatic intracranial hemorrhage (16/75), with an excellent outcome in 14 of 16 dogs. Prognosis in nontraumatic intracranial hemorrhage is reported in terms of concurrent medical conditions and the number and size of lesions.

Intracranial plasmablastic lymphoma: report of a case

患者男,32岁,蒙古国人.2009年1月因颅内占位性病变在本院手术,经多家医院会诊病理考虑为非霍奇金T细胞淋巴瘤,因送检组织较小,明确病理诊断和分型有困难.术后放疗2次(方案及剂量具体不详).10余天前患者突发右侧肢体抽搐,随即摔倒在地,意识丧失,约4 ～5 min后清醒,之后感觉嘴角麻木,右上肢无力,于2010年3月9日入住本院。

Strong 5-aminolevulinic acid-induced fluorescence is a novel intraoperative marker for representative tissue samples in stereotactic brain tumor biopsies

Stereotactic biopsies represent a routine neurosurgical procedure for the diagnosis of intracranial lymphomas and selected diffusely infiltrating gliomas. Acquisition of tissue samples that do not allow correct tumor typing and grading is, however, not uncommon. Five-aminolevulinic acid (5-ALA) has been shown to accumulate in malignant tumor tissue. The aim of this study was to prospectively investigate the clinical usability of 5-ALA for intraoperative detection of representative tissue in stereotactic tumor biopsies. Fifty consecutive patients underwent frameless stereotactic biopsy for a suspected brain tumor. 5-ALA was administered 4 h before anesthesia. Serial biopsy samples were obtained and intraoperatively checked for 5-ALA fluorescence (strong, vague, or none) using a modified neurosurgical microscope. All samples were examined for the presence of representative tumor tissue according to neuroimaging (MRI, positron emission tomography, and/or chemical shift imaging) and histopathological parameters. Visible 5-ALA fluorescence was observed in 43/50 patients (strong in 39 and vague fluorescence in four cases). At biopsy target, 52/53 samples of glioblastomas, 9/10 samples of gliomas grade III, and 14/16 samples of lymphomas revealed strong 5-ALA fluorescence. Samples with strong 5-ALA fluorescence were only observed at, but not outside the biopsy target. All tissue samples with strong 5-ALA fluorescence were representative according to our neuroimaging and histopathological criteria (positive predictive value of 100%). Our data indicate that strong 5-ALA fluorescence is a reliable and immediately available intraoperative marker of representative tumor tissue of malignant gliomas and intracranial lymphomas in stereotactic biopsies. Thereby, the application of 5-ALA in stereotactic brain tumor biopsies may in future reduce costs for operating room and neuropathology and may decrease procedure-related morbidity.

A Case of Cerebellar Lymphoma

Intracranial lymphomas are rare. Lymphomas account for 1%–3% of central nervous system tumors. Most are in the tentorium and around 50% are in the cerebral hemisphere, whereas those in the posterior fossa account for 10%–30%. A case of cerebellar lymphoma was admitted and treated in our hospital as follows.

MRI FEATURES OF CNS LYMPHOMA IN DOGS AND CATS

The magnetic resonance (MR) imaging features of central nervous system lymphoma in eight dogs and four cats are described. Intracranial lesions affected the rostrotentorial structures in six dogs and caudotentorial structures in two cats. Lesions affected the spinal cord in two dogs and in two cats. One dog and one cat with intracranial lymphoma had signs of local extracranial extension and lymphadenopathy. Lesions were considered extraparenchymal in four dogs and three cats, intraparenchymal in two dogs and one cat, and appeared to have both intra- and extraparenchymal components in two dogs. All lesions were hyperintense in T2-weighted images when compared to white matter, most were hypointense in T1-weighted images (7/12), and most were hyperintense in fluid-attenuated inversion recovery (FLAIR) images (5/9). When compared to grey matter, these lesions appear either isointense (5/12) or hyperintense (7/12) on T2-weighted images, half of them were hypointense in T1-weighted images (6/12), and most were isointense in FLAIR images (7/9). Lesion margins were usually indistinct in T2-weighted images (10/12) and had perilesional hyperintensity in FLAIR images (7/9). The majority of lesions (10/12) had abnormal meninges around the lesion and half (6/12) had generalized contrast enhancement. Mass effect was evident in all lesions. Although not specific, when combined with the history and neurologic signs, MR features aid presumptive diagnosis that should be confirmed by cytology or histopathology.

Atypical Meningioma in a Young HIV Patient

Although intracranial lymphoma is the most common primary intracranial tumor among AIDS patients, we report a 25 years old HIV positive patient with a recent onset of seizures for which he was evaluated with brain scan and was found to have a left frontal lesion with cystic necrotic appearance. Pathological examination confirmed an atypical meningioma WHO grade II. This would be the seventh case in the literature of a meningioma in an HIV positive patient. doi:10.4021/jmc40w

Cerebellar involvement in Hodgkin’s lymphoma: an atypical site of relapse

The presentation of intracranial metastases from Hodgkin's lymphoma is an infrequent event that worsens clinical outcome. A case of Hodgkin's lymphoma relapse in the cerebellum is described in a 70-year-old woman with a previously treated stage IVA Hodgkin's lymphoma. Diagnostic workup and treatment strategies for central nervous system relapses are reviewed and discussed. A combination of surgery, radiotherapy and occasionally chemotherapy remains the most appropriate approach to intracranial Hodgkin's lymphoma.

Primary dural lymphoma mimicking a subdural hematoma

Intracranial marginal zone B-cell lymphoma presenting as a dural-based mass is rare. A 45-year-old woman who had generalized tonic-clonic seizures and speech disturbance for 6 months was referred to our hospital. Radiology suggested a subdural hematoma (SDH). No improvement in the radiological findings or symptoms occurred with conservative follow-up. Therefore, she underwent a craniotomy for drainage of the suspected SDH. Intraoperatively, dural plaque-like thickening was observed, with no SDH, and a biopsy was performed. After histopathological and immunohistochemical studies, a mucosa-associated lymphoid tissue (MALT) lymphoma was diagnosed. The patient underwent radiotherapy with no postoperative complications or recurrence. Early diagnosis and treatment of primary dural lymphoma is important. Histopathological evaluation is necessary for diagnosis. MRI cannot reliably differentiate between SDH and some dural lesions that present as diffuse infiltration. Therefore, these differential diagnoses should be considered.

Atypical Meningioma in a Young HIV Patient

Although intracranial lymphoma is the most common primary intracranial tumor among AIDS patients, we report a 25 years old HIV positive patient with a recent onset of seizures for which he was evaluated with brain scan and was found to have a left frontal lesion with cystic necrotic appearance. Pathological examination confirmed an atypical meningioma WHO grade II. This would be the seventh case in the literature of a meningioma in an HIV positive patient. J Med Cases. 2010;1(2):39-41 doi: https://doi.org/10.4021/jmc40w

In Vitro Interactions Between Antifungals and Methotrexate Against Aspergillus spp.

Methotrexate has been widely used in the treatment of osteosarcoma, intracranial lymphomas and leukemia. However, patients are also at high risk of opportunist pathogens such as Aspergillus spp. infection for their deeply depressed immunity. The optimal choice of antifungal agents during the infection of Aspergillus for these patients is necessary to be explored. In this study, we investigated the interactions between antifungals and methotrexate against Aspergillus in vitro. A total of 23 clinical isolates of Aspergillus spp. were studied. Microdilution checkerboard technique was performed to evaluate the interaction of methotrexate with voriconazole, itraconazole, terbinafine and amphotericin B. The highest rate of synergy was obtained for the combination of terbinafine and methotrexate, which exhibited synergy against 60.9% (14/23) of strains. No interaction was detected for the combinations of methotrexate plus itraconazole or amphotericin B against 95.7% (22/23) or 100% of strains, respectively. Although voriconazole exhibited indifferent against 87% (20/23) of strains when combined with methotrexate, antagonism effect was found against 13% (3/23) of strains. The positive interactions of terbinafine and methotrexate were also certified by disk diffusion assay. In addition, we observed the morphological changes for the interaction of methotrexate with terbinafine against Aspergillus. Further inhibition and distortion of growth were found after the combination of terbinafine and methotrexate compared with the drugs treated alone. Clinical studies are warranted to further elucidate optimal treatments for the immunocompromised patients with Aspergillus infection.