Steroid-induced remission in primary malignant lymphoma of the central nervous system

A case of primary malignant lymphoma of the central nervous system is described in which lesions seen on computed tomography scans disappeared, and clinical remissions occurred with the administration of corticosteroids. The tumor affected the region of the fourth ventricle and parietal and frontal lobes. In our patient, three remissions occurred over a span of 24 months, each in conjunction with corticosteroid administration. This supports other observations that steroids alter the natural history of primary malignant lymphoma of the central nervous system, leading to both radiologic and clinical remissions.     

Outcome of primary central nervous system lymphoma--a study of 32 patients.

The outcomes in 32 cases of histologically diagnosed primary central nervous system lymphoma were investigated. The 1-, 2-, and 5-year survival rates were 54, 36, and 8%, respectively. Good outcome was indicated by extensive surgical removal with 50-Gy irradiation and lower ages. 61% of patients receiving radiation therapy suffered recurrence within 1 year. The incidence of multiple lesions increased at recurrence. These lesions were almost all remote from the initial site in the brain, occurring more frequently in the central part of the supratentorial regions near the ventricle. Multiple lesions recurred more rapidly than single lesions. Longer survival times were indicated by a long tumor-free period after initial treatment. Extensive surgical removal results in long survival times for patients with a localized single tumor in the early stage. Radiochemotherapy should be given as part of the initial treatment.     

Primary malignant lymphoma of the central nervous system

The occurrence of primary malignant lymphomas of the central nervous system in a population of approximately 1 million people has been analyzed retrospectively. Over a 12-year period, about 1000 tumors of the central nervous system were registered. Among these, 22 were primary malignant lymphomas; incidence, 1.83 per million per year. Thirteen of these tumors were of high-grade malignancy and nine were of low-grade malignancy (Kiel classification of non-Hodgkin lymphomas). The majority of the tumors were B-cell lymphomas, and there were no Hodgkin lymphomas. Neither spinal nor meningeal lymphomas occurred. Nine patients were treated only surgically, whereas 11 received postoperative irradiation or chemotherapy, or both. Of the latter patients, six are alive and well at the time of writing. The importance of making a correct diagnosis preoperatively is stressed, inasmuch as radical operation is unfavorable for these patients. A better prognosis is obtained with combinations of irradiation and chemotherapy.     

High-dose methotrexate for non-AIDS primary central nervous system lymphoma. Report of 13 cases

Thirteen patients with primary lymphoma of the central nervous system (CNS) were treated with high-dose intravenous methotrexate (MTX), 3.5 gm/sq m, followed by calcium leucovorin rescue, at 3-week intervals, for three cycles. Eleven patients subsequently received radiation therapy to the whole brain, 30 to 44 Gy. Before radiation therapy, eight patients responded completely and four partially; there was one non-responder. The median Karnofsky score before high-dose MTX therapy was 60 and increased to 90 after treatment. Five of the eight complete responders reached a Karnofsky rating of 100. The three longest responders (one of whom received MTX only) were without recurrence of their disease at 29+, 32, and 32+ months posttherapy. The median response period is 9+ months. The median survival time from the date of the first MTX treatment is 9+ months, and the three longest survival times are 29+, 32+, and 54+ months. All patients received corticosteroids in either unchanging or diminishing dosages during therapy. It is concluded that primary CNS lymphoma is sensitive to high-dose MTX, which provides a safe and easily administered adjuvant to radiation therapy for this neoplasm.     

Treatment of primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin's lymphoma that is typically confined to brain, eyes, and cerebrospinal fluid without evidence of systemic spread. The prognosis of patients with PCNSL has improved during the past decade with the introduction of high-dose methotrexate with or without whole brain radiotherapy. However, despite recent progress, results following treatment are durable in few patients, and therapy can be associated with late neurotoxicity. PCNSL is an uncommon tumor, and no phase III trial has been completed so far, leaving many questions about its optimum first-line and salvage treatments unanswered. This review summarizes the literature regarding the treatment of PCNSL in immunocompetent patients.     

A study of primary central nervous system lymphoma in northern India

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is rare and accounts for 1 to 2% of all lymphomas. There are conflicting reports about the rise in incidence of PCNSL cases in the last two decades; this has largely been attributed to an increase in incidence of AIDS and other immunosuppressive states in some studies. This study was undertaken to view the trend of PCNSL at the Postgraduate Institute of Medical Education and Research, Chandigarh, which is a referral hospital in northern India. METHODS: The PCNSL cases from our surgical material of 15 years (1985-1999) were reviewed. Slides were examined independently by three histopathologists. Immunophenotyping was done on paraffin-embedded tissue using indirect immunoperoxidase technique. RESULTS: Out of a total of 3,325 intracranial tumors diagnosed during this period (1985-1999), there were 40 cases (1.2%) of PCNSL; gliomas accounted for 1,531 cases (46.04%). The age ranged from 24-75 years with the sex ratio (M:F) being 2:1. HIV serology, available in 14 cases, was negative in all. The parietal lobe was the most common site of involvement. Diffuse large cell lymphoma was the most common morphological type. Immunohistochemistry could be done in 31 cases; 28 cases were found to be B-cell type whereas 3 cases were T-cell type. No statistically significant increase was seen on comparing the number of cases at 5-year intervals. CONCLUSION: This study revealed that there has been no significant increase in PCNSL cases over the last 15 years.     

原发性中枢神经系统淋巴瘤MRI表现

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的MRI表现。方法回顾性分析29例接受MR平扫+增强检查,且经手术病理证实,具有完整临床资料的PCNSL病变的MRI表现,观察病灶的部位、大小、形态、边缘轮廓、瘤周水肿、占位效应、MRI平扫信号特点,重点观察增强后病灶的典型及非典型强化特点。结果 29例中单发者16例,多发者12例(30个病灶),共46个病灶,弥漫浸润型1例。肿瘤好发于胼胝体、基底节区等深部脑组织(35/46,76.09%)。瘤周水肿多为中度(15/46,32.61%)或重度(27/46,58.70%),未见出血及钙化。肿瘤T1WI多呈等低信号(30/46,65.22%)或等信号(14/46,30.43%),T2WI多呈等信号(14/46,30.43%)或等高信号(29/46,63.04%),DWI多为高信号(42/46,91.30%)。增强扫描表现出多种强化方式:典型强化方式包括均匀一致团块状或结节状强化、"裂隙征"、"蝶翼征"、"皮层下刻痕征"、以及"卫星灶征";不典型强化方式包括环形强化、"开环样"强化、4脑室匍匐贴壁生长病灶,沿软脑膜弥漫分布粟粒结节样病灶。结论 PCNSL的MRI表现具有一定的特征性,对PCNSL的诊断及鉴别诊断具有重要的价值,但确诊仍有赖于病理。     

Radiological features of primary central nervous system lymphoma

Radiological features of 12 cases of primary malignant lymphoma of the central nervous system with histological confirmation were reviewed. Ten patients had primary intracranial lymphoma, and two spinal. Angiography yielded no specific findings. CT appearances varied widely. Any positive relationships were not found between histologic types of tumors and patterns of contrast enhancement in the present cases as well as in those reported previously. Gd-DTPA was used in two patients. All lymphomas showed low to isointensity signals on T1-WI and high signal intensities on rho-WI and T2-WI. Although MRI gave no additional informations to those on CT with regard to the differential diagnosis, it proved to be a very accurate, non-invasive method of providing better delineation of tumor on multiplanar basis. Use of Gd-DTPA improved quality of T1-WI by markedly enhancing the tumor. It is generally difficult to distinguish primary from secondary spinal lymphoma, but two spinal epidural lymphomas in the present series were considered primary, as we could detect no other lesion elsewhere in the body. We expect that MR imaging would lead to an improvement in the detection of lesion in the paravertebral area.     

Two cases of primary intracranial malignant lymphoma

Two cases of primary intracranial malignant lymphoma are reported. Case 1 was a 65-year-old female who was operated on for a left frontoparietal tumor in 1966 at our hospital, and the tumor was diagnosed as reticulum cell sarcoma histologically. Irradiation was also done. Thereafter, she had had no serious trouble except for slight right hemiparesis. In March of 1982, right hemiparesis became worse and disturbance of consciousness also appeared. CT scan and left carotid angiogram revealed a left fronto-temporoparietal tumor. Subtotal removal of the tumor was performed on the 1st of April. The tumor was diagnosed as malignant lymphoma, of large cell type. Chemotherapy was started postoperatively and marked decrease of enhanced area was observed in the CT scan taken on the 9th of July. But the patient showed melena and was diagnosed as having rectal adenocarcinoma histologically by biopsied specimen. Case 2 was a 48-year-old male who had been suffering from left facial palsy and vertigo since August, 1981. Those symptoms gradually progressed. On the 4th of December, he was admitted to our clinic. On admission, neurologically, multiple cranial nerve involvement was noticed such as of the IIIrd, IVth, Vth and VIth nerves bilaterally, and the VIIth, IXth, Xth and XIth cranial nerves on the left side. Right hemihypalgesia, thermohypesthesia, dysmetria, dysdiadochokinesis, and cerebellar taxia were also seen. CT scan and left vertebral angiogram revealed a tumor in the pons and IVth ventricle. On December 8th, suboccipital craniectomy was performed, but almost all of the tumor seemed growing under the floor of the IVth ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary malignant lymphoma of the central nervous system. Results of treatment of 11 cases and review of the literature

Eleven patients with primary malignant lymphoma of the central nervous system (CNS) were treated at the Medical College of Wisconsin Affiliated Hospitals between 1964 and 1984. Three patients had a prior history of immunosuppressive therapy following renal transplantation. All patients had biopsy-proven disease and 10 of the 11 were treated with external radiation therapy. The doses to the primary tumor ranged from 34 to 59.4 Gray (Gy). Actuarial (life-table) survival rate was 82% at 1 year and 43% at 3 years. No recurrence was seen after 13 months. Eighty-six reports totaling 693 cases of primary malignant lymphoma of the CNS were found in the literature. Of these, 308 cases were treated with a combination of surgery and irradiation. Overall survival at 5 years for those patients who received more than 50 Gy compared with less than 50 Gy to the primary tumor was 42.3% versus 12.8% (p less than 0.05). Twenty-one patients survived longer than 5 years. Late relapse was notable, with 10 (47.6%) of 21 tumors recurring between 5 and 12.5 years after diagnosis. Based on this review, a minimum of 50 Gy radiation to the primary tumor is recommended. While no statement regarding the efficacy of craniospinal irradiation or chemotherapy can be made in view of the small numbers, the use of craniospinal irradiation and/or systemic chemotherapy should be considered for future trials.     

Granulomatous angiitis of the central nervous system--a case with recurrent intracerebral hemorrhage.

Granulomatous angiitis of the central nervous system occurred in a 43-year-old male presenting with recurrent intracerebral hemorrhage confirmed by computed tomography. A surgical specimen obtained at hematoma removal revealed granulomatous angiitis. Systemic investigation found no underlying cause for the hemorrhage. Although the incidence is very low, this condition should be considered in cases of recurrent intracerebral hemorrhage with unknown cause. When surgery is indicated, biopsy of the brain and leptomeninges should be obtained, because involvement of the leptomeninges has occurred in virtually all autopsy cases.     

Characteristic features of malignant lymphoma with central nervous system involvement

BACKGROUND: Cerebral lymphoma is becoming increasingly common. METHODS: We reviewed the records of all our patients with non-Hodgkin's lymphoma (NHL) seen from April 1987 to August 1996 in our institute. Our analysis of these patients with lymphomatous central nervous system (CNS) involvement documents the clinical features, histology, and prognostic factors in CNS lymphoma. RESULTS: A total of 351 cases of NHL were treated in our institute. CNS lymphoma was found in 58 of 351 (16.5%) patients in our series. Forty-nine of 58 (84.5%) patients with CNS involvement also had systemic disease. Primary CNS lymphomas were detected in nine patients. Leptomeningeal infiltration was seen in 31 of 58 patients, whereas intracerebral infiltration was detected in 28 patients. Initial symptoms of CNS involvement included severe headache, muscle weakness, and other neurological signs. Malignant cells were detected in 32 of 132 studies in the cerebrospinal fluid examination. In the nine patients with primary CNS lymphoma, the median survival time was 16.5 months (range, 4-28 months). The overall median survival of the 58 CNS lymphoma patients was only 13.4 months (range, 1 to 32 months). CONCLUSIONS: Because prophylactic treatment was only successful in systemically well-controlled patients, control of systemic lymphoma seems to be of great importance.     

原发性胃恶性淋巴瘤20例术前误诊探讨

笔者回顾性分析20例胃原发性恶性淋巴瘤的诊治情况。20例术前经X线或胃镜检查及活检均误诊。手术切除20例，其中根治性切除15例，姑息性切除5例。术后并发腹腔脓肿1例，食道空肠吻合口瘘1例。虽然胃原发性恶性淋巴瘤误诊率较高，但其预后比胃癌好，所以治疗时,应积极行根治性手术，辅以化疗或放疗。     

Prognosis in postoperative discitis. A retrospective study of 111 cases.

111 cases of postoperative discitis during 1968-1986 were analyzed retrospectively. The diagnosis was confirmed by lumbar tomography. Low back pain appeared at an average of 16 days postoperatively. Laboratory findings were of minor value in the diagnosis since elevated ESR, white blood cell count, and body temperature were inconstant findings. Compared with a matched control group, there was a higher incidence of chronic low back pain and vocational handicap in the discitis patients. There was no difference in the consumption of analgetics, the subjective evaluation of the final outcome, spinal mobility or neurologic findings.     

Isolated primary central nervous system lymphoma arising from the optic chiasm

A 58-year-old previously healthy woman rapidly developed progressive bilateral visual loss. Magnetic resonance imaging revealed a bulging appearance of the optic chiasm, with homogeneous enhancement after gadolinium administration, which suggested an optic glioma or inflammatory disease. In the absence of (para)clinical clues for a specific diagnosis despite extensive investigation, a biopsy of one optic nerve was performed, resulting in a diagnosis of non-Hodgkin B-cell lymphoma. There was no evidence of any other ocular or systemic involvement, therefore the conclusion was that this immunocompetent patient had a primary central nervous system lymphoma isolated in the anterior visual pathway. Treatment included two cycles of polychemotherapy (rituximab, methotrexate, carmustine, etoposide, methylprednisolone), followed by autologous peripheral blood stem cell transplantation and rituximab plus cytarabine consolidation therapy. Subsequently, the patient exhibited significant improvement in vision, and was still disease-free at the 1-year follow-up examination. The aim of the present paper was to provide well-documented clinical, radiological, and intraoperative features of isolated primary malignant lymphoma arising from the anterior visual pathway. A better recognition of this rare pathological entity is necessary for clinicians who may encounter similar presentations, as prompt management is crucial for both a visual and vital prognosis.     

54例原发性结直肠非霍奇金淋巴瘤的预后因素分析

目的:探讨原发性结直肠非霍奇金淋巴瘤(NHL)临床病理因素与预后的关系.方法:回顾1978年5月至2003年3月收治的54例原发性结直肠非霍奇金淋巴瘤资料,采用单因素和多因素方法分析临床、病理、治疗方式与预后的关系.结果:5年总生存率为58.5%,10年总生存率为55.7%.单因素分析显示有无B症状、血清乳酸脱清酶(LDH)水平、病理免疫表型(T/B)、是否行根治性手术切除和临床分期与结直肠非霍奇金淋巴瘤的预后相关,多因素分析显示病理免疫表型(T/B)、临床分期与预后相关.结论:病理免疫表型(T/B)和临床分期是原发性结直肠非霍奇金淋巴瘤独立的不良预后因素.T细胞性淋巴瘤预后较差,临床分期越晚预后越差.     

Effectiveness of DeVIC chemotherapy for recurrent primary central nervous system lymphoma

Six cases with recurrent or refractory primary central nervous system lymphoma were treated with a new chemotherapeutic regimen "DeVIC (dexamethasone, VP16, ifosfamide, carboplatin)". Five recurrent cases had a remission period for an average of 18 months after initial treatment, but relapse occurred. One refractory case had no response after initial treatment. Then these 6 cases were treated with 1-3 courses of DeVIC chemotherapy at intervals of 4 weeks. Two cases achieved complete remission, and 3 cases attained partial remission (response rate was 83%). One case showed no response after 1 course of DeVIC chemotherapy. However, in all cases re-relapse occurred 1-5 months after remission, and only 1 case is still alive. DeVIC chemotherapy produced a high response rate for recurrent central nervous system lymphoma, but re-relapse occurred after only a few months. The establishment of maintenance chemotherapy is required.