Primary low grade B-cell lymphoma of the dura in an immunocompetent patient

Primary central nervous system lymphomas (PCNSL) represent only 1% of all non-Hodgkin's lymphomas (NHLs). A 66 year-old woman was hospitalized due to multiple episodes of syncope and seizures which occurred the week before admission to the hospital. A computerized tomography (CT) scan of the brain showed a right parietal lesion suggesting a subdural effusion. The patient was operated and a dural lesion extending to the epidural space and cerebral cortex was excised. Histologic findings suggested diagnosis of a low-grade lymphoma of the mucosa associated lymphoid tissue (MALT) type. The patient was treated with radiation therapy and has now completely recovered 12 months after surgery.     

Grade II oligodendroglioma localized to the corpus callosum

The presence of a primary central nervous system (CNS) neoplasm within the corpus callosum generally portends a grave prognosis. Common pathologies encountered include glioblastomas and primary CNS lymphomas. In contrast, World Health Organization grade II oligodendroglial tumors demonstrating loss of heterozygosity at 1p and 19q are generally less aggressive, often responding favorably to chemotherapy. The authors present a case of a primary brain tumor isolated to the corpus callosum diagnosed as a grade II oligodendroglioma. A 52-year-old woman presented with new-onset generalized seizure. Magnetic resonance imaging (MRI) revealed a non-contrast-enhancing lesion with associated edema and regional mass effect. The patient underwent a craniotomy and subtotal resection of the lesion using an endoscopic port. Pathological examination revealed a grade II oligodendroglioma. Molecular analysis identified 1p and 19q deletion as well as MGMT promoter methylation. The patient subsequently underwent adjuvant radiation therapy with an excellent response. We present, to our knowledge, the first report of a grade II oligodendroglioma isolated within the corpus callosum with the characteristic molecular features of this tumor type. Histopathologic diagnosis is essential to appropriately guide therapy of callosal tumors.     

Imaging Analysis of Primary Malignant Intracranial Lymphoma

OBJECTIVE To investigate the images characteristics of primary malignant intracranial lymphoma.METHODS Retrospective study was conducted on CT/MRI imaging characteristics of 9 cases with primary malignant intracranial lymphoma.RESULTS The patients had lesions mostly in the supratentorial region, including the parts of deep white matter, para-ependymal regions, and corpus callosum. The shapes of the lesions were round or irregular. CT scan showed equal or slightly high density of the tumor images, compared with the normal tissue in the brain.The TIWI of MRI scan on the tumor showed low signal and the T2WI showed equal or slightly high signal. The MRI signals were homogenous. Cystic lesion, calcification, and hemorrhage were rarely seen in MRI. Edema around tumor and its occupational effect was lessened. Edema around tumor shown in MRI was not in accordance with the true volume of the tumor mass. Enhanced scan on the lesions showed homogenous enhancement, and the pia mater invaded and/or the spread along ependyma. CONCLUSION Images of primary malignant intracranial lymphoma have specific characteristics that are useful in its diagnosis and differential diagnosis.     

Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients

To avoid an unnecessary extend of surgery in primary central nervous system lymphoma (PCNSL), the diagnosis should be suspected after MRI.Pre-treatment MRI examinations of 100 immunologically competent patients with biopsy-proven PCNSL were evaluated. All patients had T2- and T1-weighted images with contrast enhancement. Diffusion-weighted MRI (DW-MRI) was available in 15, proton-MR-spectroscopy (¹H-MRS) in four patients.The number of lesions ranged from one (n=65 patients) to eight (n=1) with a mean of 1.7. The most frequent locations were the cerebral hemispheres (n=66), the basal ganglia (n=27) and the corpus callosum (n=24). In the 65 patients with a solitary lesion, hemispheric lesions were most frequent (n=23) followed by corpus callosum (n=18). Contrast enhancement was found in all but one patient. ¹H-MRS revealed a uniformly pathologic pattern of metabolite concentrations in all patients.Characteristic imaging features of PCNSL are contrast-enhancing lesions with a diameter of at least 15 mm in contact with the subarachnoid space. DW-MRI and proton spectroscopy may aid in differential diagnosis.     

A primary CNS lymphoma in spontaneous remission for 3.5 years after initial detection of the lesions by MRI

We describe the clinicopathological features of a patient with an autopsy-proven primary CNS lymphoma, who had a relatively long remission period after onset. A 61-year-old man experienced disorientation and gait disturbance. A ventriculoperitoneal shunt operation was performed, based on the diagnosis of hydrocephalus due to aqueductal stenosis, after which his symptoms subsided. Three months later, T₂-weighted magnetic resonance imaging (MRI) revealed high-intensity lesions in the corpus callosum and tectum. However, he remained asymptomatic for the next three and a half years, during which periodic MRI studies constantly detected the lesions. At the age of 65 years, he suffered respiratory and consciousness disturbances, and his general condition gradually deteriorated. MRI studies disclosed that the callosal lesion had spontaneously disappeared, whereas the tectal lesion had developed to become an enhanced linear lesion with a tendency to spread on the dorsal side of the brain stem. Examination of the cerebrospinal fluid disclosed a markedly elevated β₂-microglobulin content, and a tentative diagnosis of malignant lymphoma was made. Steroid pulse therapy had little effect, and the patient died four years after presentation. Examiaation at autopsy disclosed a malignant, large B-cell lymphoma that had diffusely infiltrated the cerebrum and brain stem. In the corpus callosum, a small number of residual lymphoma cells were seen around the vessels. Therefore, the initially detected lesions in the corpus callosum and tectum might have been attributable to lymphoma, and the unusual clinical and radiological features of this case provide further information that might aid in diagnosis and help to ensure prompt treatment.     

中枢神经系统恶性淋巴瘤

中枢神经系统恶性淋巴瘤是一种罕见神经系统肿瘤。本文报告１２例，其中男８例，女４例。单发１１例，其中幕上７例，幕下４例；多发１例。临床表现无特殊性。好发部位幕上为额叶，幕下为小脑半球，且常累及脑室，胼胝体，硬膜和大脑镰。肿瘤病理组织学表现同身体其他部位恶性淋巴瘤，治疗采用手术和放疗为主，化疗为辅的原则，但病人预后差。     

Imaging analysis of primary malignant intracranial lymphoma

OBJECTIVE To investigate the images characteristics of primary malignant intracranial lymphoma. METHODS Retrospective study was conducted on CT/MRI imaging characteristics of 9 cases with primary malignant intracranial lymphoma. RESULTS The patients had lesions mostly in the supratentorial region, including the parts of deep white matter, para-ependymal regions, and corpus callosum. The shapes of the lesions were round or irregular. CT scan showed equal or slightly high density of the tumor images, compared with the normal tissue in the brain. The TIWI of MRI scan on the tumor showed low signal and the T2WI showed equal or slightly high signal. The MRI signals were homogenous. Cystic lesion, calcification, and hemorrhage were rarely seen in MRI. Edema around tumor and its occupational effect was lessened. Edema around tumor shown in MRI was not in accordance with the true volume of the tumor mass. Enhanced scan on the lesions showed homogenous enhancement, and the pia mater invaded and/or the spread along ependyma. CONCLUSION Images of primary malignant intracranial lymphoma have specific characteristics that are useful in its diagnosis and differential diagnosis.     

Extranodal marginal zone B-cell lymphoma of MALT-type of the lung: single-center experience with 12 patients

The lung is a relatively rare site for mucosa-associated lymphoid tissue (MALT) lymphomas: we report the largest available single-center series of patients with this presentation. From August 1992 to October 2000, 12 patients with untreated primary low-grade MALT lymphoma of the lung were submitted either to chemotherapy alone (n = 8), surgery alone (n = 2) or surgery plus chemotherapy (n = 2). At diagnosis, 6 (50%) were asymptomatic and 6 (50%) had nonspecific pulmonary symptoms. The most common radiologic findings were a pulmonary infiltrate (7 cases) and a mass lesion (5 cases). Histological diagnosis was obtained with transbronchial lung biopsy/bronchoalveolar lavage (BAL) (6 cases), with transthoracic needle biopsy (1 case), or an open thoracotomy (5 cases). All patients had stage IE. All 12 (100%) achieved complete remission; 3 (25%) local recurrences were observed. The global 6-year survival rate was 100% with a relapse-free survival rate of 50%. In conclusion, these data underline the diagnostic utility of BAL and the therapeutic efficacy of a chemotherapeutic strategy based on regimens such as N-CVP in the context of localized MALT lymphoma of the lung.     

Radiologic and pathologic features of a posttransplantation primary central nervous system lymphoma demonstrating Epstein-Barr virus-positive Hodgkin lymphoma

Posttransplantation lymphoproliferative disease is the most common malignancy in T-cell immunocompromised patients and often involves extranodal sites including the central nervous system (CNS). Primary posttransplantation CNS lymphomas are rare and, accordingly, are poorly characterized. Herein, for the first time, we describe the radiologic and pathologic features of a primary CNS posttransplantation lymphoproliferative disease that reveals Epstein-Barr virus-positive Hodgkin lymphoma. The lesion showed characteristic Hodgkin-Reed Sternberg cells with a typical immunophenotype, accompanied by numerous nonneoplastic B cells and T cells demonstrating classic Hodgkin lymphoma within the brain. The 65-year-old patient was treated by reduction of immunosuppression and cerebral radiation therapy and remains in remission 18 months after the initial diagnosis.     

原发性胃肠道淋巴瘤的诊断与治疗

目的探讨原发性胃肠道淋巴瘤的临床特点及诊治方法。方法1983年至2004年在北京大学第一医院外科接受治疗的原发性胃肠道淋巴瘤患者35例,对其临床病理资料及预后进行回顾性分析。结果本组原发性胃肠道淋巴瘤术前诊断率仅为20.0%;18例胃淋巴瘤中16例接受手术治疗,其中12例行根治性切除;17例肠道淋巴瘤均手术治疗,其中14例行根治性切除;Ann Ar-bor临床分期为:ⅠE期14例,ⅡE期10例,ⅢE期1例,ⅣE期10例。MALT淋巴瘤与非MALT淋巴瘤在年龄、性别及发病部位及早晚分期上差异无显著性。MALT淋巴瘤组手术根治率(92.9%)高于非MALT淋巴瘤(68.4%),MALT淋巴瘤组淋巴结转移率(42.9%)明显低于非MALT淋巴瘤组(84.2%)。结论胃肠道淋巴瘤临床表现缺乏特异性,术前诊断率低;胃淋巴瘤与肠淋巴瘤在性别、年龄、手术根治性及临床分期方面无明显区别;MALT淋巴瘤组确诊时分期较早,手术根治率高,淋巴结转移率低,预后优于非MALT淋巴瘤组。外科手术是诊断和治疗原发性胃肠道淋巴瘤的主要方法。     

Frame-based stereotaxy in a frameless era: current capabilities,relative role,and the positive- and negative predictive values of blood through the needle

Introduction In the modern era of frameless stereotaxis (FL), the role of frame-based (FB) stereotactic needle biopsy is evolving. Methods Retrospective review of prospective database of 106 lesions in 91 consecutive patients undergoing FB stereotactic needle biopsy with a systematic “geologic core” technique by a single surgeon. Diagnostic accuracy was calculated comparing biopsy diagnosis with final pathology in 11 patients who underwent subsequent surgical resection. All instances of intra-operative bleeding through the needle were prospectively noted and compared with post-biopsy CT scan. Lesions were classified as risky for FL technique if they were (1) infratentorial or pineal, (2) within 10 mm of the circle of Willis or root of the Sylvian fissure, or (3) within 10 mm of deep cerebral veins. Results Diagnostic yield was 94%. Diagnostic accuracy was 91%. Of 18 lesions involving the corpus callosum, 13 (72.2%) were GBM 2 were anaplastic astrocytoma, and 1 each were found to be anaplastic oligodendroglioma, primary central nervous system lymphoma (PCNSL) and tumescent MS. Of 25 multifocal lesions, malignant primary brain tumor was diagnosed in 17 (68%) (11 GBM, 3 PCNSL, 2 anaplastic ologodendroglioma, and 1 anaplastic astrocytoma). Mortality was 0%. Three patients developed temporary neurologic deficits and one had permanent deficit. Absence of persistent blood through the biopsy needle had a negative predicative value of 98.8% for subsequent neuroimaging blood >5 mm diameter. According to our criteria, 80% of patients would have been candidates for FL biopsy. Conclusions Stereotactic biopsy is an effective, safe and important technique for histologic diagnosis of brain lesions, particularly for multifocal and corpus callosum lesions. Post-biopsy CT can be safely reserved for patients who demonstrate persistent bleeding through the biopsy needle. FB stereotaxy remains an important technique for the 20% with small or deep seated lesions or when it is advantageous to avoid an incision, a burr hole or general anesthesia.     

淋巴瘤单发骨病变的18F－FDGPET／CT影像学分析

目的：研究原发性骨淋巴瘤与单发继发性淋巴瘤骨髓浸润的18 F －FDG PET／CT 影像学表现,探讨18 F－FDG PET／CT 对原发性骨淋巴瘤的诊断及鉴别诊断价值。方法：回顾性分析经病理证实的25例单发骨淋巴瘤的18 F －FDG PET／CT 影像学资料。结果：25例骨淋巴瘤均为单发,其中14例位于脊柱骨,10例位于附肢骨,1例位于肋骨。15例为原发性骨淋巴瘤,10例为继发性淋巴瘤骨髓浸润。原发性骨淋巴瘤15例中非霍奇金淋巴瘤11例,霍奇金淋巴瘤4例;继发性淋巴瘤骨髓浸润10例中非霍奇金淋巴瘤7例,霍奇金淋巴瘤3例。25例骨淋巴瘤中23例 CT 表现为骨质密度异常改变,2例病变骨质密度未见明显异常改变。病变 FDG摄取不同程度增高,SUVmax范围为2．6～24．5。原发性骨淋巴瘤及继发性淋巴瘤骨髓浸润病变 SUVmax经 Mann－Whitney U 检验提示原发性骨淋巴瘤与继发性淋巴瘤骨髓浸润病变 SUVmax有差异（P ＝0．007）。结论：原发性骨淋巴瘤18 F －FDG PET／CT 影像学表现有一定的特征性,分析其表现对原发性和继发性骨淋巴瘤的诊断及鉴别诊断有一定的临床价值。     

Treatment and prognosis in a series of primary extranodal lymphomas of the ocular adnexa

Background: The aim of this study was to assess clinicopathological characteristics and outcome in a series of primary ocular adnexal lymphomas (POALs).Patients and methods: Nineteen patients with localised (stage IE) POAL were followed for a median of 96 months (24–156). The diagnosis was based on surgical biopsies followed by immunohistochemistry in 16 cases or fine-needle aspiration followed by immunocytophenotypic analysis in three cases. Twelve patients were treated with local radiotherapy (RT), five with chemotherapy (CT), and two refused further therapy after apparently radical tumour removal achieved by the diagnostic excisional biopsy.Results: The histological and immunological pattern was consistent with a diagnosis of MALT-type lymphoma (11 cases), follicular center non-Hodgkin's lymphoma (three cases), a large-cell variant of Burkitt's lymphoma (one case), and large-cell transformed MALT lymphoma (one case). Low-grade lymphoma was diagnosed in the three cases which underwent fine-needle aspiration biopsy. All of the patients achieved and maintained complete remission except for those treated with surgical excision alone (two MALT conjunctival lymphoma cases): one of these relapsed locally, the other experienced the systemic spread of a transformed diffuse large-cell lymphoma and died 72 months after diagnosis. The side effects consisted of two cases of RT-related cataract after 52 and 72 months.Conclusions: Regardless of histology, prognosis was excellent when surgery plus RT was adopted, and CT seems to be a valid alternative to RT. Surgery alone may be sub-optimal.     

Irinotecan (topoisomerase-I inhibitor) for the treatment of recurrent primary intracranial malignant lymphoma

Primary intracranial malignant lymphoma is a fetal disease with poor prognosis, and there is no effective treatment against recurrent primary intracranial malignant lymphomas. We report 3 cases of malignant lymphoma treated with irinotecan (topoisomerase-I inhibitor, camptothecin derivatives), an aromatic drug extracted from camptotheca acuminata. After the initial diagnosis, surgical resection followed by radiation therapy was performed for one cerebral, and two cerebellar malignant lymphomas. The tumors recurred 1 month, 18 months, and 18 months after the initial treatment, respectively. The former two cases were treated with additional radiation therapy and/or radiosurgery for the recurrent tumors; however, the tumors recurred again. All cases were treated finally with a combination therapy of irinotecan and cis-platinum followed by a maintenance therapy with irinotecan only. All cases showed a sharp roentgenographical response to the chemotherapy even after cumulative recurrences. One patient died of systemic infection, and another died of intracranial tumor recurrence 11 and 29 months after the initial diagnosis, respectively. Autopsies revealed multiple tumor recurrences in both these cases. The other patient died 31 months after the initial diagnosis, also due to intracranial tumor recurrences. These results indicate the usefulness of irinotecan for the treatment of recurrent primary intracranial malignant lymphoma; however, further investigation is necessary to establish a better protocol for irinotecan treatment against primary intracranial malignant lymphoma.     

Trends in primary cerebral lymphoma.

Primary non-Hodgkin lymphoma of the brain is rare, representing only 1% of all non-Hodgkin lymphomas (NHLs), but its incidence has been increasing rapidly in south-east England since 1985. Among 17,322 cases of NHL registered during the 18 year period 1973-90, there were 210 cases of primary cerebral NHL, of which 179 (86%) were diagnosed in the last third of this period, 1985-90. This increase in cerebral lymphoma is not adequately explained by improvements in the precision of diagnosis or by changes in disease coding or cancer registration practice. While there has also been a rapid increase in Kaposi sarcoma, neither immunosuppression acquired through HIV infection nor the overall trend in non-Hodgkin lymphoma can satisfactorily explain the recent increase in cerebral lymphoma, which affects all ages and both sexes similarly. Other possible causes for a true increase in cerebral lymphoma should be sought.     

Role of fluorine‐18‐fluorodeoxyglucose positron emission tomography/computed tomography in evaluating breast mucosa‐associated lymphoid tissue lymphoma: A case series

Mucosa‐associated lymphatic tissue (MALT) lymphoma of the breast is an extremely rare disease; its pathogenesis is not clear because of the rarity of disease, and the best diagnostic method has yet to be established. The metabolic behavior of this lymphoma is not still clear because only a few case reports are present in literature describing the possible role of fluorine‐18‐fluorodeoxyglucose positron emission tomography/computed tomography (18F‐FDG PET/CT) in this field. This report presents 4 cases of women with histologically proven breast MALT lymphoma who underwent 7 18F‐FDG PET/CT throughout the course of disease. All patients underwent staging PET/CT showing in all cases an FDG avid lesion corresponding to breast lymphoma; 3 patients underwent 18F‐FDG PET/CT also after chemotherapy. Our results suggest that breast MALT lymphomas are 18F‐FDG‐avid lymphomas. Positron emission tomography/computed tomography showed heterogeneous but high FDG uptake (mean maximum standardized uptake value 7.9), suggesting that it could be part of diagnostic workup and restaging process.     

Primary gastric Hodgkin's lymphoma: a case report and review of the literature

Primary gastric Hodgkin's lymphoma is a rarely encountered lesion. Most cases are observed in the course of systemic disease. Other cases have been reclassified in retrospective studies as non-Hodgkin's lymphomas, after the employment of immunohistochemistry. Some Hodgkin's lymphomas may masquerade non-Hodgkin's lymphomas, and vice versa. Therefore, an accurate diagnosis is important, as treatment and outcome differ significantly for these entities. We report a case of primary Hodgkin's lymphoma arising in the stomach of a 46-year-old male, and discuss the diagnostic approach as well as the differentials of Hodgkin's disease in this anatomic site.     

Clinical,Histopathological, Immunophenotypic and Molecular Analysis of 60 Patients with Cutaneous T-cell Infiltrates with Follow up of Indeterminate Cases to Identify T-cell Lymphoma

Diagnosis of primary cutaneous T-cell lymphomas, especially of mycosis fungoides could be difficult in early stage due to clinical and histopathological similarity to reactive inflammatory dermatoses. To assess diagnostic value of complex histological, immunophenotypic and T-cell receptor gamma gene rearrangement analysis, skin biopsy specimen and peripheral blood samples of 60 patients with suspected cutaneous T-cell lymphoma were analyzed. Our results indicate clear distinction between reactive dermatoses (benign cases, n = 31) and cutaneous T-cell lymphomas (lymphoma cases, n = 17). As definite diagnosis was not obtained in a smaller group of patients (indeterminate cases, n = 12), these patients were followed up. Repeated skin biopsy confirmed mycosis fungoides in 6/12 cases, however in 6/12 patients the diagnosis remained indeterminate. We concluded that careful and complex clinical follow up and repeated histopathological, immunophenotypic and molecular analysis is needed for an appropriate diagnosis in the assessment of early stage mycosis fungoides and uncertain clinical cases.     

颅内脂肪瘤的CT和MRI诊断

 目的 评价CT和MRI在颅内脂肪瘤诊断中的价值。方法 对经CT及MRI诊断并经手术病理证实的10例颅内脂肪瘤进行对照总结。结果 肿瘤位于胼胝体8例,第三脑室、外侧裂各1例。其中3例胼胝体脂肪瘤同时发生双侧脑室脉络丛脉脂肪瘤,罕见。CT表现均匀低密度7例,不均匀低密度3例,肿瘤伴钙化3例。MRI检查3例在TIWⅠ及T2WⅠ图像均为高信号。结论 研对颅内胼胝体等部位显示形态各异低密度,CT值在0以下,或伴钙化肿块;MRI在T1WⅠ和T2WⅠ图像显示与颅外脂肪相同的高信号肿块,可作为CT、MRI诊断颅内脂肪瘤的重要表现。     

Gastric low-grade MALT lymphoma, high-grade MALT lymphoma and diffuse large B cell lymphoma show different frequencies of trisomy.

Gastric MALT lymphoma is a distinct entity related to Helicobacter pylori gastritis. Some studies suggest a role for trisomy 3 in the genesis of these lymphomas, but they mainly focused on low-grade MALT lymphoma. Gastric MALT lymphoma, however, comprises a spectrum from low- to high-grade cases. Furthermore, its exact relation to primary diffuse large B cell lymphoma (DLBCL) of the stomach is not clear. We applied in situ hybridisation (ISH) with centromeric probes on 43 samples of 39 patients with primary gastric lymphoma (13 samples with low-grade MALT lymphoma, 25 with high-grade MALT lymphoma and five with DLBCL) to detect numerical aberrations of 10 chromosomes. ISH was performed immunohistochemically on nuclei isolated from paraffin-embedded resection tissue and on whole paraffin sections using immunofluorescence. In six of 13 low-grade MALT lymphomas trisomy was detected (46%) and mostly involved chromosome 3 (33%). In high-grade MALT lymphomas, trisomies were found in 16 of 25 cases (64%), mainly involving chromosomes 12 and 18. Trisomy 3 was present in only 13% of these cases. Of five DLBCL, only one showed trisomy. Nine of the 16 aberrant high-grade MALT lymphomas (56%) showed trisomy of more than one chromosome per case vs two of six for low-grade cases. In lymphomas with separate low- and high-grade tumour components some trisomies were detected in both components, whereas others occurred only in the high-grade tumour cells. This supports the hypothesis that high-grade MALT lymphomas can develop from a low-grade type and that this progression is accompanied by the acquisition of more genetic aberrations. However, trisomy 3 probably does not play a major role in this progression.