1/10. Chronic lymphocytic leukaemia presenting with central nervous system involvement.A 68-year-old man presented with hemiparesis, lymphocytosis, and cerebral lesions on MRI. flow cytometry of blood, bone marrow and cerebrospinal fluid showed B-CLL lymphocytes with bright CD20 expression, sIg, and absence of CD23 antigen. fluorescence in situ hybridisation showed trisomy 12 in 50% of analysed peripheral mononuclear cells. The patient died 6 months after the diagnosis. Rapidly progressive and fatal course of the disease was consistent with known bad prognostic significance of CD20 bright expression and trisomy 12.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/10. Primary anaplastic large cell lymphoma of the central nervous system.central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL), and in children only isolated cases have been reported, mainly as secondary CNS involvement. A case of fatal primary ALCL of the brain in a 13-year-old white boy is reported. magnetic resonance imaging of the brain showed decreased absorption in T1- and T2-weighted image showed a hyperintense signal in the right parietal lobe and 2 masses in the right frontal lobe. A frontal lobe biopsy showed a pleomorphic neoplasm diffusely infiltrating the brain parenchyma and composed of large cells with bizarre, often polylobated or horseshoe-shaped nuclei. Immunohistochemical stains showed diffuse strong positivity for CD30, anaplastic lymphoma kinase protein (ALK-1), p80, leucocyte common antigen, CD45RO (UCHL1), and focal staining for epithelial membrane antigen. Immunostainings for cytokeratins, monocyte-macrophage, and B-cell markers were negative. Epstein-Barr virus latent membrane protein was not detected. To the best of our knowledge, there is only 1 case of primary ALCL of the brain in childhood previously reported in the literature. Before the biopsy, both cases were clinically misdiagnosed as mycobacterial CNS infection. Therefore, primary ALCL should also be included in the differential diagnosis when a mycobacterial CNS infection is suspected in pediatric patients; a careful cytological evaluation of the cerebrospinal fluid or cerebral biopsy are essential for an accurate diagnosis.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/10. cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions.OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, israel. patients: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, arnold-chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/10. Lyme borreliosis mimicking central nervous system malignancy: the diagnostic pitfall of cerebrospinal fluid cytology.We report two children with acute loss of neurological functions and signs of an increased intracranial pressure. Imaging techniques ruled out space occupying lesions, whereas CSF cytology indicated CNS involvement of a non-Hodgkin lymphoma in the form of abnormal lymphocytic pleocytosis with malignancy criteria fulfilling lymphoid cells. CSF protein electrophoresis and borrelia burgdorferi serology revealed neuroborreliosis which was successfully treated with antibiotic therapy. The malignancy mimicking cytology is based on a blastoid transformation of B- and t-lymphocytes due to the antigenic stimulus of B. burgdorferi infection. Lymphoid cells in the CSF of a patient with acute or chronic neurological symptoms raise the differential diagnosis of inflammatory etiology versus CNS lymphoma. Monomorphism and higher quantity of the lymphoid cells point to CNS lymphoma. A lower quantity and polyclonal pattern of lymphoid cells associated with an elevated protein fraction caused by intrathecal immunoglobulin synthesis suggest an inflammatory etiology.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/10. Primary intestinal aspergillosis after high-dose chemotherapy and autologous stem cell rescue.Primary invasive aspergillosis of the gut is a rare event and is associated with high mortality. We report for the first time on a patient who had isolated aspergillosis of the small bowel after autologous stem cell transplantation. diagnosis of invasive aspergillosis of the gut was based on abdominal pain, galactomannan antigenemia and isolation of aspergillus fumigatus from the stool and was later confirmed by pathohistologic examination. No other site of invasive aspergillosis was evident. The patient was successfully treated with early surgery and combination antifungal therapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/10. Primary Ki-1 (anaplastic large cell) lymphoma of the brain and spinal cord.The authors report a case of primary Ki-1 lymphoma of the brain. The patient was a 4 1/2-year-old black girl who presented with a 4- and 5-day history of headaches, nausea, vomiting, neck stiffness, and difficulty in walking. Computed tomography (CT) scan of the brain showed two discrete densities in the left occipital lobe and in the brain stem. magnetic resonance imaging (MRI) showed multiple densities scattered over the brain surface and brain stem. Microscopically, the tumor was an anaplastic neoplasm that diffusely infiltrated brain parenchyma. The neoplastic cells were large with amphophilic cytoplasm, large nuclei with irregular nuclear contours and prominent nucleoli. A high mitotic rate including atypical mitotic figures was noted. Immunohistochemical stains showed diffuse strong positivity for CD30 and moderate focal staining for epithelial membrane antigen. Leukocyte common antigen, cytokeratin, neuron specific enolase, monocyte/macrophage and B- and T-marker stains were negative. The histology was characteristic for Ki-1 large cell lymphoma. Cytologic examination of cerebrospinal fluid (CSF) demonstrated similar neoplastic cells. This is one of the first reports of this variant in the pediatric population.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/10. "Polyphenotypic" tumors in the central nervous system: problems in nosology and classification.In recent years, there is increasing recognition of polyphenotypic high-grade malignancies in the non-central nervous system (CNS) tumor literature. Some of these tumors have been regarded as variants of primitive neuroectodermal tumor (PNET) or as extrarenal malignant rhabdoid tumors (MRTs). This report concerns two posterior fossa neoplasms, both of which displayed a "polyphenotypic" expression of neural, epithelial, myogenic, and glial markers, including synaptophysin, neurofilament, vimentin, glial fibrillary acidic protein, S-100, neuron-specific enolase, desmin, S antigen, MIC2, cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. One tumor showed complex intercellular junctions, cytoplasmic intermediate filaments, well-developed rough and smooth endoplasmic reticulum and golgi apparatus, cilia, and neurosecretory granules. The other neoplasm showed pools of glycogen, desmosomes, and tonofilaments. The histological and ultrastructural appearances were inconsistent with glioma, PNET, meningioma, ependymoma, choroid plexus carcinoma, sarcoma, germ cell tumor, and other tumors in the world health organization classification. Although the polyphenotype raises the issue that these may represent variants of MRT or the atypical teratoid-rhabdoid tumor, the morphologic findings in the two cases were very dissimilar. Our two cases underscore the problems in nosology and classification of polyphenotypic tumors of the CNS. This is particularly significant, as therapeutic protocols for PNET, MRT, and non-CNS polyphenotypic tumors are different. We review the literature on polyphenotypic tumors and reiterate the difficulties in precise classification of these complex tumors.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
8/10. immunohistochemistry of medulloepithelioma and neural tube.immunohistochemistry profiles of medulloepithelioma (from two 2 1/2-year-old girls who had cerebral medulloepitheliomas and a 35-week postconceptional female infant with congenital posterior fossa tumor) and neural tube are compared. Microscopically, the tumors contained a medulloepitheliomatous component, manifested as tubular epithelial structures lined by pseudostratified columnar epithelium delineated by well-defined basement membranes. In all cases, glial and neuronal differentiation were noted to differing extents. The medulloepitheliomatous components did not exhibit glial fibrillary acidic protein, neuron-specific enolase, or S-100 protein reactivity. Neurofilament, cytokeratin, and epithelial membrane antigen were focally present in one case. Extensive nestin immunopositivity was confined to the basal cell layer of the epithelium, leaving the luminal surface unreactive or slightly reactive. These cells also displayed a reactivity to vimentin and to microtubule-associated protein type 5 similar to that of cells of the primitive neural tube. The similarity between the immunohistochemical profile of medulloepithelioma and that of neural tube epithelium suggests a possible reexpression of that component of the genome responsible for neural tube growth and differentiation in medulloepithelioma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/10. Phenotypic diversity of neurofibromatosis 2: association with plexiform schwannoma.AIMS: Plexiform schwannoma (PS) is a rare variant of benign schwannoma characterized by a multinodular and plexiform growth pattern. In contrast to plexiform neurofibroma. PS is not associated with neurofibromatosis 1 (NF-1; von Recklinghausen's disease) and has no propensity for malignant transformation. The purpose of this study was to clarify the relationship between PS and the entities of neurofibromatosis 2 (NF-2; bilateral acoustic neurofibromatosis) and schwannomatosis. methods AND RESULTS: Six cases of PS associated with NF-2 or meningioma were retrospectively studied clinicopathologically and immunohistochemically. Four cases of PS were found among the patients with NF-2, and all of these had multiple PSs; three cases also had multiple schwannomas of the spinal nerve roots and two of these had meningioma. Two other patients had meningioma, but not NF-2. Four patients were male and two were female. The ages ranged from 18 to 52 years (mean 29.6 years). Histologically, PS showed the histological features that have been previously described, i.e. schwannoma composed of a predominant Antoni A-type component with a plexiform growth pattern. Immunohistochemically, the tumour cells were positive for S100 protein. Each nodule was surrounded by perineural cells which were positive for epithelial membrane antigen. CONCLUSIONS: It is important to recognize that PS could be associated with NF-2 or meningioma. The combination of PS and meningioma may be a 'formes frustes' of NF-2, and is clinically overlapped with schwannomatosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/10. Quiescent nasal T/NK cell lymphoma manifested as primary central nervous system lymphoma.A 57-year-old man was diagnosed as primary T/NK-cell central nervous system lymphoma (CNSL) with intraocular involvement. However, review of a surgical specimen taken three years before for chronic paranasal sinusitis revealed an overlooked nasal T/NK cell lymphoma (TNKL), which showed similar histomorphology and immunophenotype with the CNS disease. Another patient, a 43-year-old woman, was initially diagnosed as a rare primary leptomeningeal T-cell lymphoma with ocular manifestation. Three years later, an isolated nasal TNKL emerged. Immunohistochemical and cytogenetic studies confirmed the same nature of the CNSL and the nasal TNKL. The nasal TNKLs of both patients had a strong expression of CD3, CD56, and Epstein-Barr virus antigens, but features of angiodestruction and mucosal ulceration were absent. We propose that: 1. a locally silent "quiescent" form of nasal TNKL may exist; and 2. a thorough examination and even blind biopsy of the nasal cavity is indicated when primary T/NK-cell CNSL is diagnosed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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