1/105. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings.Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/105. Hypophyseal non-Hodgkin's lymphoma presenting with diabetes insipidus: a case report.We report the case of a 64 year old male patient with a history of ischemic heart disease who underwent surgery for an abdominal mass. The histological diagnosis was highly malignant non-Hodgkin's lymphoma. After surgery the patient was admitted to our Department and received 6 courses of chemotherapy according to the COP schedule, followed by radiotherapy to the left upper abdominal region and ipsilateral lung base. The patient achieved partial remission. One month later he began to complain of left axillary lymphadenomegaly, polydipsia and polyuria. A NMR brain scan showed a hypophyseal mass. The patient was treated with DDAVP and chemotherapy with the PRO-MACE protocol; the polyuria and lymphadenomegaly disappeared and the size of the hypophyseal mass reduced markedly. The clinical picture was, therefore, attributed to a hypophyseal localization of the non-Hodgkin's lymphoma, which is a very rare manifestation of lymphomatous spread to the central nervous system. Our case is also interesting because it shows that a favorable outcome can be obtained with chemotherapy, provided that the latter is sufficiently aggressive. This is not necessarily the case with radiotherapy which may also be followed by late and severe neurologic sequelae.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/105. Combined central retinal artery and vein occlusion in a child with systemic non-Hodgkin's lymphoma.PURPOSE: To report on a case of systemic non-Hodgkin's lymphoma and unilateral combined central retinal artery and vein occlusion. METHOD: We examined a 14-year-old boy who experienced a sudden unilateral visual loss five months after the initial diagnosis of systemic non-Hodgkin's lymphoma. RESULT: Visual loss was due to combined central retinal artery and vein occlusion in association with tumoral optic nerve involvement. CONCLUSION: Although very rare systemic non-Hodgkin's lymphoma may present with central retinal artery and vein occlusion prior to overt central nervous system involvement.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/105. Primary cardiac lymphoma. A case report and review.Primary cardiac lymphoma is classically defined as an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. However, over the last few years, this definition has been extended to include other localizations on condition that these are clearly less important then a cardiac site, that must remain the first, during the illness course, and the most important for its entity. PCL is extremely rare in immunocompetent patients, accounting for 1.3% of all cardiac tumours and 0.5% of all extranodal lymphomas, but it has been encountered with increasing frequency in patients with AIDS or other severe immunodepressive syndromes. PCL is difficult to diagnose, especially during the early stage of the disease, because of its non-specific clinical manifestations, the limited possibility of using non-invasive diagnostic techniques, and difficulties or delays in applying invasive methods. The malignancy of its histotypes and its delicate location are responsible for its rapid and frequently unfavourable evolution. Successful treatment, which is mainly based on anthracycline-containing polychemotherapies, is heavily dependent on an early diagnosis. After a general review of the literature, the authors describe the clinical case of a patient with a PCL that had a secondary central nervous system location, treated with polychemotherapy and autologous peripheral blood stem cell transplantation. Emphasis is placed on the fact that it is more difficult to eradicate the disease from the central nervous system than from the heart.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
5/105. Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin's lymphoma.Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system with an acute clinical onset and a wide variability in severity and outcome. It usually follows a viral infection or an immunization and is thought to be immuno- mediated. We report a case of ADEM with a dramatic clinical onset in an autologous peripheral blood stem cell transplant (PBSCT) recipient for non-Hodgkin's lymphoma who developed the neurologic syndrome 12 days after PBSC reinfusion. This is the first report of ADEM in the setting of autologous PBSCT, a therapeutic procedure performed with increasing frequency in a wide variety of hematologic and solid malignancies.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/105. Lack of CD 29 (beta1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis.Intravascular Lymphomatosis (IL) is a rare and usually aggressive form of non-Hodgkin's lymphoma characterized by the growth of neoplastic cells within vascular lumina that usually presents with skin or central nervous system (CNS) involvement. The mechanism(s) for the selective intravascular growth of this neoplasm remain(s) unexplained. We now report clinical and immunohistologic data on surgical material from 6 cases of IL; in 4 of 6 cases, autopsies were performed. Our IL cases shared the following features: (1) B-cell lineage; (2) lack of skin involvement at presentation; (3) aggressive behavior; and (4) lack of extravascular lymphomatous masses; in addition, 1 case had an associated gastric low-grade MALT lymphoma. We studied by immunohistochemistry formalin-fixed, paraffin-embedded sections with monoclonal antibodies to molecules known to be involved in lymphocyte and endothelial adhesion phenomena, that is, CD29 (beta1 integrin subunit), CD43 (leukosialin), CD44 (H-CAM), CD54 (ICAM-1), embryonal N-CAM (e-NCAM), and EMA (episialin). In all cases, the surfaces of IL aggregates reacted for CD44 but were consistently negative for CD29; also absent was CD54. Conversely, the integrity of the endothelial cells was underscored by their even reactivity for CD29, CD44, and CD54. Given that CD29 is currently regarded as critical for lymphocyte trafficking in general and for transvascular migration in particular, and CD54 is also involved in transvascular lymphocyte migration, we conclude that their consistent absence in IL may contribute to its intravascular and disseminated distribution pattern. The rather frequent association of IL with various conventional lymphomas is known; yet, one of our cases appears to be the first report of IL associated with a low-grade MALT lymphoma.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/105. Isolated primary hepatic lymphoma in a patient with acquired immunodeficiency syndrome.Non-Hodgkin lymphoma (NHL) of the B-cell type is the second most common neoplasm in patients with human immunodeficiency virus (hiv) infection after Kaposi sarcoma (KS). The majority of cases of NHL in patients with acquired immunodeficiency syndrome (AIDS) involve extranodal sites; most frequently the gastrointestinal tract (GIT) and the central nervous system (CNS). Hepatic NHL in patients with AIDS was first described by Reichert et al in 1983 in an autopsy series. It usually presents with multiple large hepatic masses and involvement of other abdominal organs or lymph nodes. The authors present a case of primary hepatic NHL in a patient with AIDS, presenting with innumerable small intrahepatic masses without the involvement of any other organs.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/105. Unusual extra-axial central nervous system involvement of non-Hodgkin's lymphoma: magnetic resonance imaging.The MR imaging findings in a patient with non-Hodgkin's lymphoma with unusual involvement of the sella, pituitary stalk and left parasellar region are reported here. On the basis of the MR imaging findings, the initial differential diagnosis included invasive pituitary adenoma, a granulomatous lesion and en plaque meningioma. Trans-sphenoidal biopsy of the sellar mass showed chronic inflammatory changes and the patient was initially treated for tuberculosis. Because follow-up imaging showed the lesion to be progressive, a biopsy was done of an enlarged right inguinal lymph node. This revealed non-Hodgkin's lymphoma.- - - - - - - - - - ranking = 4keywords = nervous system (Clic here for more details about this article) |
9/105. guillain-barre syndrome in a patient with non-Hodgkin's lymphoma.We describe a case of guillain-barre syndrome (GBS) in a patient with non-Hodgkin's lymphoma (NHL). A 21-year-old woman with a newly diagnosed stage IV high-grade lymphoma (precursor T-cell NHL according to the R.E.A.L. classification) developed flaccid quadriparesis and bilateral facial diplegia after three weeks of treatment with vincristine, daunorubicin, L-asparaginase and prednisolone. The clinical course and neurological examination were consistent with GBS. Despite treatment with intravenous immunoglobulins her neurological symptoms progressed. plasmapheresis was therefore initiated followed by intravenous immunoglobulins. After partial remission of neurologic symptoms, induction chemotherapy with cyclophosphamide and cytarabine was continued without any further complication. Three months later, the lymphoma was in complete remission. GBS has been described in Hodgkin's disease and after bone marrow transplantation but is rare in NHL. In patients with NHL who develop neurological symptoms, drug toxicity and nervous system infiltration are the leading cause of neuropathology, but GBS should be considered in the differential diagnosis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/105. Intravascular lymphomatosis: contribution of cerebral MRI findings to diagnosis.Intravascular lymphomatosis (IL) is a rare variant of non-Hodgkin's lymphoma with an unusual predilection for the central nervous system (CNS). Most cases are not diagnosed until postmortem because of variable clinical presentation and nonspecific laboratory findings. neuroimaging findings vary widely and range from diffuse involvement of the deep white matter to infarct-like lesions. Cerebral magnetic resonance imaging (MRI) may show parenchymal and meningeal gadolinium enhancement. The authors describe brain MRI findings of linear, punctate, and patchy enhancement suggestive of CNS IL in two patients confirmed by brain biopsy/histologic studies. High index of clinical suspicion and careful interpretation of MRI (including gadolinium contrast studies) may contribute to premortem diagnosis and early intervention of this often-missed disease.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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