Primary angiitis of the central nervous system mimicking dissemination from brainstem neoplasm: a case report

BACKGROUND: Diagnosis of PACNS is very difficult because of the variety of clinical manifestation, especially neurologic and MRI findings. We report the case of a 68-year-old woman with PACNS. CASE DESCRIPTION: Her clinical symptoms mimicked malignant brainstem neoplasm disseminated to third and fourth ventricles and cervical spinal cord. Brain biopsy could not be correctly diagnostic. In spite of a trial of high-dose steroids, repeated MRI showed an increasing number of lesions. Two weeks after surgery, she died of respiratory dysfunction. Postmortem examination suggested PACNS, after consideration of histories, laboratory and radiologic findings, and the lack of systemic disease. CONCLUSION: Although brain biopsy represents the gold standard measure, combination with several other examinations might be necessary to arrive at an early and definitive diagnosis. PACNS should be considered the differential diagnosis of intracranial tumors.     

Primary angiitis of the central nervous system

Primary angiitis of the central nervous system (PACNS) is a rare disorder that must be differentiated from secondary CNS vasculitides, reversible cerebral vasoconstriction syndromes, and other vascular disorders. Because of the rarity of PACNS, no controlled trials have been performed, thereby precluding an evidence-based approach to therapy. PACNS has been considered to have three major subsets, as defined by clinical, laboratory, angiographic, and pathologic findings: granulomatous angiitis of the central nervous system (GACNS), benign angiopathy of the central nervous system (BACNS), and atypical PACNS. Approximately 5% of cases may present as a tumor-like mass lesion. Because the subset of patients with GACNS has a more guarded prognosis, treatment with high-dose glucocorticoids and cyclophosphamide is necessary. All patients with atypical PACNS should be treated with high-dose glucocorticoids initially. The use of additional immunosuppressive agents depends on the severity of the initial presentation, the ability to achieve remission with glucocorticoid monotherapy, and the occurrence of relapse upon glucocorticoid tapering. PACNS mass lesions can regress completely with immunosuppressive therapy, and therefore, surgical excision may be avoided. Patients with BACNS should be treated with a calcium channel blocker (eg, verapamil) with or without a short course of glucocorticoids. These patients should not receive additional immunosuppressive therapies such as cyclophosphamide. This reflects the fact that BACNS is now more correctly considered as a reversible cerebral vasoconstriction syndrome (RCVS) and not as a true vasculitis. However, a patient initially diagnosed with RCVS that demonstrates incomplete resolution of angiographic findings after 3 to 4 months should be re-evaluated for alternative diagnoses, including PACNS.     

Biopsy findings in primary angiitis of the central nervous system

Primary angiitis of the central nervous system (PACNS) is a form of vasculitis restricted to the brain and spinal cord, with protean clinical manifestations and often slowly progressive course. Outcomes vary, ranging from spontaneous resolution to rapid decline and death. Diagnosis of PACNS is based on angiography and/or biopsy. We reviewed surgical biopsies from 46 patients (53 biopsies) with PACNS, including 25 men and 21 women (median age 46, range: 25 to 84 y) and correlated the findings with relevant clinical parameters. Biopsies (51 brain, 2 spinal) were diagnostic of vasculitis in 29 (63%) patients. Three morphologic patterns of vasculitis were observed: acute necrotizing (n=4, 14%); purely lymphocytic (n=8, 28%); and granulomatous (n=17, 58%), 8 associated with deposition of beta-A4 amyloid. Biopsies not diagnostic of PACNS (n=17, 37%) showed nonspecific gliosis (53%), mild perivascular mononuclear inflammation (18%), and parenchymal ischemic damage/infarct (18%). All positive biopsies were among those directed to an imaging abnormality (targeted biopsies) and biopsies including leptomeninges were more often positive than those that did not. Thus, where possible, a targeted biopsy that includes leptomeninges is recommended to maximize diagnostic potential. No statistically significant differences in outcome were noted among the 3 histopathologic groups or when comparing biopsy positive versus biopsy negative PACNS groups. Overall the outcomes were relatively favorable, with only 14% mortality or severe morbidity at 1.14 years (mean) after biopsy.     

Primary rhabdomyosarcoma of the central nervous system: case report

A case of a primary rhabdomyosarcoma that appeared to be arising from the filum terminale in a 48-year-old man is reported. The histological diagnosis of rhabdomyosarcoma was supported using immunocytochemical methods. Twenty-two cases of primary rhabdomyosarcoma of the central nervous system previously reported in the literature are reviewed briefly. The possible origin of these tumors from neuroectoderm is discussed.     

Primary central nervous system T-cell lymphoma. Case report

Primary central nervous system (CNS) T-cell lymphoma is extremely rare. The present case report provides immunocytochemical evidence for a cerebellar CNS T-cell lymphoma. The patient underwent surgery followed by radiation therapy and is alive and well 36 months postoperatively. The clinical and pathological features of primary CNS T-cell lymphoma as well as diagnostic measures and treatment options are discussed, together with a compilation of all previous case reports of primary CNS T-cell lymphomas.     

Primary central nervous system mucosa-associated lymphoid tissue lymphoma--case report

A 55-year-old woman presented with an extremely rare primary central nervous system mucosa-associated lymphoid tissue (MALT) lymphoma manifesting as seizure and was subsequently diagnosed with dural MALT lymphoma in the cranium. Magnetic resonance imaging revealed a left frontal dural mass with peritumoral edema. Histological examination of the dural mass confirmed MALT lymphoma and revealed infiltration of small cells with irregular nuclear borders and expression of a B cell marker (CD20) but absence of CD5, CD10, CD23, and cyclinD1. Reactive T-cell infiltration was also seen. Subsequently, local irradiation (40 Gy/20 fractions) was performed. Magnetic resonance imaging showed complete remission just after irradiation was completed. There was no evidence of systemic MALT lymphoma. There has been no recurrence for 3 years without additional therapy.     

Primary central nervous system lymphomatoid granulomatosis: a case report

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated multisystem lymphoproliferative and granulomatous disease histologically characterized by angiocentric and angiodestructive cellular infiltrate. LYG usually involves the lungs, skin, kidneys and brain, but isolated central nervous system LYG has been reported rarely. We describe a 57-year-old man with histologically proved LYG confined to the brain. The patient complained of visual disturbance, and was admitted to our hospital. Gadolinium-enhanced T1-weighted MRI showed irregular, partly cystic and lobulated enhancement in the left occipital lobe, suggesting the presence of possible neoplastic lesions such as glioma, malignant lymphoma or metastatic brain tumor. We performed surgical resection of the lesion. Histological and immunohistochemical examinations of the tumor showed angiocentric polymorphic infiltration and granulomatous reaction, consisting of CD3-positive small lymphocytes and CD20-positive large atypical cells. Subsequently, in situ hybridization study for EBER (Epstein-Barr virus encoded small RNA) was performed and proved that the large atypical cells were positive for EBER. These findings were compatible with LYG. We emphasize that the possibility of LYG should be considered in the differential diagnosis of any ring-like enhancing mass lesions on MR images, though less specific for LYG. Histopathological examination is indispensable for the final diagnosis and the choice of an optimal treatment strategy.     

Primary central nervous system T-cell lymphoma. Case report.

A 46-year-old male presented with a rare primary non-Hodgkin's lymphoma of the central nervous system of T-cell lineage, localized primarily in the right parietal region. There was no evidence of acquired immunodeficiency syndrome. Biopsy of the tumor allowed immunohistochemical confirmation of the diagnosis. Irradiation and chemotherapy were given, and the patient has remained well for 24 months. The clinical manifestations, management, and outcome of T-cell lymphoma are very similar to those of B-cell lymphoma.     

Primary central nervous system lymphoma

Opinion statement Standard therapy does not exist for the treatment of primary central nervous system lymphoma. This is because of low patient numbers, slow accrual to clinical trials, and the absence of phase III randomized clinical trials. Neuro-oncologists agree that whole brain irradiation alone is no longer the best treatment option; treatment should include high-dose methotrexate chemotherapy, and treatment strategies must combine efficacy without untoward toxicity. However, beyond this, there is no widespread consensus. Significant neurocognitive toxicity has been associated with combined chemotherapyradiation regimens, especially in elderly patients. This has prompted many to defer radiation therapy in all patients except those that refuse chemotherapy, those that have a partial response to chemotherapy, or those that have disease progression through chemotherapy. There is supporting evidence for this approach; however, confirmatory data from multicenter clinical trials and 5-year survival rates are unavailable. This underscores the importance of ongoing clinical trials.     

Primary central nervous system lymphoma

Primary lymphoma of the central nervous system (CNS), including reticulum cell sarcoma, microglioma, and histiocytic lymphoma, represents less than 1% of all primary brain tumors. In the last 10 years, this tumor has tripled in frequency in the nonimmunosuppressed population. By 1991, the tumor will be the most common neurological neoplasm by virtue of the increase in sporadic occurrence and in the acquired immunodeficiency syndrome (AIDS) population. Three percent of AIDS patients will develop this tumor either prior to AIDS diagnosis or during their subsequent course. In addition to acquired immunosuppression, patients with inherited disorders (such as Wiskott-Aldrich syndrome, severe combined immunodeficiency, and X-linked immunodeficiency) and other acquired disorders of the immune system are predisposed to the development of CNS lymphoma. Immunological studies have suggested a role for Epstein-Barr virus in the production of this tumor. Although subtypes exist, non-Hodgkin's lymphoma of the CNS most commonly consists of histiocytic cells or large immunoblastic cells bearing B cell surface markers in close proximity to the lateral and third ventricles. Sixty percent of these deposits are multiple, and subarachnoid invasion is seen in one-quarter of patients. Vitreous involvement of the eye occurring prior to and during the course of CNS lymphoma has been noted in up to 25% of patients. The involvement of multiple areas of the neuraxis, the eye, and multiple intracranial sites often occurs in the absence of obvious systemic lymphoma. Therapeutic trials of brain radiation therapy are associated with median survivals of less than 1 year. Uniform complete responses of intracranial deposits are recorded following chemotherapy with high-dose intravenous methotrexate, CHOP (cyclophosphamide, hydroxydaunomycin/doxorubicin, Oncovin (vincristine), and prednisone), high-dose cytosine arabinoside, and intra-arterial methotrexate with barrier modification.     

Primary central nervous system lymphoma

Opinion statement There is no class I evidence for any therapeutic option in primary central nervous system lymphoma (PCNSL). When possible, patients should be included in clinical trials. The role of surgery is restricted to stereotactic biopsy in order to gain material for histopathologic diagnosis. Radiotherapy alone is associated with a median survival of no more than 1.5 years; cure is exceptional. However, in patients aged younger than 60 years, cure is the therapeutic aim. Polychemotherapy based on high-dose methotrexate with deferred radiation results in long-term survival in most of these patients and possibly cure in a substantial fraction of these patients. With regard to chemotherapy in PCNSL, the following must be considered: 1) the most efficient drug in PCNSL is methotrexate at a dosage of at least 1.5 g/m² per single dose; 2) methotrexate alone will lead to complete remission in only some patients, whereas the combination of methotrexate with other drugs is more efficient; and 3) the value of additional intraventricular chemotherapy and the necessity of “consolidation” radiotherapy after response to chemotherapy are not yet defined. For patients aged older than 60 years, no curative regimen with acceptable toxicity has yet been established. The combination of radiotherapy with methotrexate-based chemotherapy leads to severe long-term neurotoxic sequelae, ie, cognitive dysfunction, in most older patients and in some patients aged younger than 60 years.     

Primary central nervous system lymphoma

A retrospective analysis of 21 cases of primary central nervous system (CNS) lymphoma is reported. All patients presented with a solitary mass in the supratentorial region. None had previously received immunosuppressive therapy. Neuroradiological studies included technetium-99m-pertechnetate brain scanning in eight cases, cerebral arteriography in all 21 cases, and computerized tomography (CT) in 14 cases. The characteristic features were increased uptake in brain scans, mass effect in arteriograms, and marked contrast enhancement on CT scans. Abnormal tumor vessels were occasionally seen on arteriography, and subtraction films were usually required to appreciate tumor stain. All patients underwent craniotomy, and histological studies of the tumors showed a diffuse type of lymphoma in all cases. Immunoglobulin testing was performed in 19 cases and a monoclonal spike was verified in 10, suggesting a B cell origin. All patients were followed until their death except one who was still alive 12 months from onset of symptoms. Therapy included subtotal resection in all 21 cases, whole-brain irradiation in six cases, chemotherapy in two cases, and a combination of whole-brain irradiation and chemotherapy in nine cases. Three different forms of chemotherapy were used. The results suggest that chemotherapy is an important addition to subtotal resection and whole-brain irradiation in the treatment of primary CNS lymphoma.     

颅内原发性黑色素瘤的诊断及治疗

目的 探讨颅内原发性黑色素瘤的诊治方法。方法 分析１９６８年以来收治的６例颅内原发性黑色素瘤的临床表现、诊治方法和疗效。结果 临床表现无特异性,ＣＴ及ＭＲＩ均难以明确诊断。肿瘤全切除１例。在碚切除３酌,部分切除１例,活检１例。诊断颅内原发性恶性黑色素瘤３例,脑膜黑色素瘤３例。随访５例,３例恶性黑色瘤患者死亡,２例脑膜黑色素瘤３例,随访５例,３例恶性黑色素瘤鹗２死亡,２例脑膜黑色素瘤１例长期卧床,１     

Isolated central nervous system angiitis first presenting as spontaneous intracranial hemorrhage

Two cases of isolated central nervous system angiitis presenting as spontaneous intracranial hemorrhage are reported. The usefulness and limitations of cortical/leptomeningeal biopsy in this condition is described. Emphasis is placed on the clinical awareness of this unusual association to ensure early recognition and prompt management with immunosuppressive therapy.     

Primary T-cell type lymphoma of the central nervous system: a case report

A case of T cell type primary lymphoma of the CNS is reported. A 50-year-old man was admitted because of speech and memory disturbance and dyscalculia. He had suffered from uveitis of both eyes two years before admission. Neurological examination revealed motor aphasia, finger agnosia, dyscalculia and memory disturbance. CT scan showed a mass lesion which was enhanced irregularly by the contrast medium in the left temporoparietal region. CAG confirmed the presence of a left temporoparietal avascular mass. Total removal of the tumor was performed through the left temporoparietal craniotomy. Histopathological diagnosis of the tumor was malignant lymphoma (diffuse, large cell type). Immunological study with tumor cells in the cerebrospinal fluid revealed that the tumor was T cell type malignant lymphoma. After postoperative whole CNS irradiation and intrathecal methotrexate injection, his preoperative symptoms disappeared. No evidence of the tumor recurrence is seen under CT scan one year and two months after the tumor resection. Relationship between the cell type of the tumor and CT findings is discussed.     

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.     

Granulomatous angiitis of the nervous system: a case report of long-term survival

In 1977, a 61-year-old man developed an occipital hemorrhage, and a biopsy specimen revealed granulomatous angiitis of the nervous system. No concurrent infection or systemic vasculitis was present. After initial treatment with steroids and cyclophosphamide, the patient did well on chronic steroids alone for 4 years. He then independently stopped taking the steroids and suffered a second hemorrhage. Steroids were reinstated, and he did well for 3 more years before he experienced a third hemorrhage. Intravenous steroids were given acutely and tapered to a chronic maintenance dose. Although granulomatous angiitis of the nervous system usually causes death within weeks to months of the appearance of symptoms, the patient is still alive more than 12 years since the tissue-proven diagnosis. This unusually long survival is attributed to the continued use of steroids, even when the patient is asymptomatic.