Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional.
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PMID:Primary brain lymphoma presenting as Parkinson's disease. 1121 45

Intravascular lymphomatosis (IL) is a rare entity only recently included in lymphoma classification, whose main feature is the exclusive or predominant growth of neoplastic cells within blood vessels. The vast majority of the patients affected by IL belong to the 7th or 8th decade of life and present with skin rash or CNS diffuse necrotic or demyelinating lesions. Case report. SS, a 13-year-old girl, was admitted to a Neurosurgery Unit because of endocranic hypertension, where, after CT and MRI documenting a IV ventricle 3 cm diameter tumor, she was submitted to complete tumor excision: extemporary diagnosis was suggestive of medulloblastoma. When referred to us she had persistent fever with normal blood and spinal fluid cultures. Whole CNS MRI did not give evidence of residual or metastatic disease while CSF cytology showed only pleiocytosis. Treatment was started according to our ongoing protocol for medulloblastoma with pre-radiation chemotherapy. Before delivering radiotherapy (RT), upon review of histologic specimens, the definitive diagnosis of IL B-phenotype was made. The girl was re-admitted and, after a complete re-staging, chemotherapy was intensified according to our schedule for high-grade B-cell lymphoma and CNS was irradiated up to a total dose of 25 Gy. She remained alive in continuous complete remission at 21 months after diagnosis. The case here reported is unique for age, tumor presentation, and, so far, favourable outcome, in spite of the delayed histological diagnosis.
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PMID:Intravascular lymphomatosis (IL) in a child mimicking a posterior fossa tumor. 1134 80

Primary vaginal non-Hodgkin's lymphomas (NHL) are rare, and are clinically difficult to differentiate from inflammatory diseases or vaginal cancer. Here, we present such a case in a 74-year-old woman complaining of fever and difficulty with urination. Pelvic examination revealed a tumor involving most of the vaginal wall, and pelvic MRI demonstrated vaginal wall thickening. A biopsy of this lesion confirmed NHL (diffuse large B-cell lymphoma), and the patient was admitted. Abdominal CT and MRI detected a vaginal tumor, and Ga scintigraphy confirmed accumulation in the pelvis, but no abnormalities were seen in other areas. Therefore, the patient was diagnosed as having NHL at clinical stage IB with low-intermediate risk (international prognosis index) (LDH 1,309 IU/L). The patient underwent three courses of CHOP therapy followed by radiotherapy, and complete remission was achieved. Primary vaginal NHL often affects women younger than 50 years of age, and abnormal hemorrhage is the initial symptom in many cases. There have been a number of reports of long-term survival following appropriate early chemotherapy and radiation therapy, suggesting that early diagnosis and treatment based on vaginal biopsy findings greatly influence the prognosis.
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PMID:[Primary lymphoma of the vagina]. 1152 48

Mucosa-associated lymphoid tissue (MALT) lymphomas are low-grade B-cell neoplasms that occur in a variety of extranodal sites. Only rarely has this entity been discovered as a primary tumor involving the dura of the cavernous sinus. We report the case of a 46 year old woman who presented with a mass involving the cavernous sinus that was originally diagnosed as a meningioma by MRI. However, at surgery the mass was found to be an extranodal marginal zone B-cell lymphoma of the MALT type. The patient underwent partial excision of the lesion followed by radiation. There are only seven other cases of marginal zone B-cell lymphoma involving the intracranial dura reported in the literature. These patients were females who presented with intracranial lesions thought to be consistent with meningioma by preoperative radiographic imaging. MALT lymphomas of the dura, as their counterparts in other organs, appear to have favorable clinical outcomes and excellent long-term prognoses with local therapy alone.
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PMID:Extranodal marginal zone B-cell lymphoma of malt type involving the cavernous sinus. 1169 33

A 54 year old man is described with signs compatible with ocular myasthenia gravis and an apparent excellent response to pyridostigmine. Subsequent clinical progression and further investigation suggested the presence of an inflammatory brain stem lesion, which responded to corticosteroid therapy. Clinical relapse, including the development of central neurogenic hyperventilation, led to a brain stem biopsy, confirming a diagnosis of B cell lymphoma. This case illustrates the propensity of primary CNS lymphoma (PCNSL) to mimic other conditions. Brain MRI is mandatory in presumed "test negative" ocular myasthenia with atypical clinical findings. Spontaneous regression of PCNSL or response to corticosteroids is common and should not mitigate against the diagnosis. Histopathological confirmation should ideally be made before starting therapy, as this may obscure or delay the correct diagnosis. Although PCNSL is rare, it must be considered in all patients with brain stem syndromes, and in all patients 50 years or older with contrast enhancing focal lesions.
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PMID:B Cell lymphoma of the brain stem masquerading as myasthenia. 1179 84

We describe a case of primary anaplastic diffuse large-cell lymphoma arising in the central nervous system (CNS). Primary CD30-positive anaplastic diffuse large B-cell lymphoma of the brain is very rarely reported. Given that this tumor is immunohistochemically heterogeneous, polymerase chain reaction (PCR) and Epstein-Barr virus (EBV) analysis of tumor DNA are essential techniques for early and accurate histological diagnosis in these CD30-positive cerebral lymphoma cases. We report an early CD30- and EBV-positive anaplastic diffuse large B-cell lymphoma in the CNS that was diagnosed not only from the immunohistochemical study and MRI findings, but also from the genotype confirmations. This tumor was documented to have EBV episomes of monoclonal origin by PCR analysis of immunoglobulin gene rearrangement.
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PMID:Detection of Epstein-Barr virus DNA and expression of CD30 antigen in primary anaplastic diffuse large B-cell lymphoma of the brain. 1190 74

We report a 71-year-old man with intravascular malignant lymphomatosis who showed high serum LDH and urinary disturbance for one year before manifesting dementia. High serum LDH was found at a health check at age 70. Two months later, he had an onset of backache and urinary retention. MRI of the spinal cord was unremarkable. One year later, he showed decline of mental activities and was admitted to our hospital. He was agitated and confused. However cranial nerve palsy or limb weakness was not noted. The MRI of the brain showed T2-high signal in bilateral occipital, right temporal lobe and the left insular cortices. The abdominal CT scan showed swelling of the adrenals on both sides. Adrenal biopsy revealed diffuse large B cell lymphoma. He developed respiratory distress and he died two months after the admission. Post mortem examination revealed intravascular and extravascular proliferation of lymphoma cells in most of the internal organs including adrenals, spleen, liver and the kidneys. In the brain, the laminar necrosis was seen in the left occipital cortex and hemorrhagic infarctions were noted in the insular and temporal cortices and the medial temporal cortex. Sacral spinal cord showed necrosis of the gray matters and loss of myelinated fibers in the white matter. Intravascular proliferation of the lymphoma cells were also seen in the vessels of the brain and the spinal cord. This patient suggests the importance of survey for intravascular malignant lymphomatosis, when high serum LDH and myelopathy of lumbosacral area are seen.
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PMID:[A patient with intravascular malignant lymphomatosis presenting subacute dementia one year after sustained urinary retention and high serum LDH activity from the onset]. 1208 Jun 10

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.
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PMID:A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement. 1208 50

We report 2 adults with a past history of radiation therapy to the head for malignancy (one with primary B-cell lymphoma confined to the skull and the other with multiple hemangioendotheliomas) who developed episodes consistent with migraine with and without aura. In addition to more typical migraine attacks and beginning many years after their radiation therapy, both patients have experienced infrequent, stereotyped, prolonged, reversible neurologic deficits associated with headache, occasional seizures, and striking, transient, cortical gadolinium enhancement of the posterior cerebral gyri on MRI. Interictal MRI brain scans show stable abnormalities consistent with the patients' previous radiation therapy. The neurologic deficits often progressed over a few days, sometimes lasted weeks, and completely resolved. Electroencephalograms did not show epileptiform activity. Thorough investigation showed no residual or recurrent tumor and no recognized cause for the patients' attacks. We postulate a causal relationship between the patients' remote radiation therapy and their prolonged, strokelike migraine attacks. Radiation-induced vascular changes could provoke the episodes, with or without an underlying migraine diathesis. Recognition of this syndrome can help avoid invasive testing.
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PMID:Reversible, strokelike migraine attacks in patients with previous radiation therapy. 1267 84

We report a rare case of malignant lymphopma of the cranial vault. A 71-year-old woman developed an asymptomatic subcutaneous mass on the scalp. The skin over the lesion was normal and the mass was firm and attached to the skull. Neurological examination revealed no abnormalities. Plain CT scans showed a high-density lesion that was widely attached to the skull and the surface of the skull was slightly irregular. About 25 days later, enhanced CT showed that the mass was well enhanced and extended into the cranium, and decalcification of the skull adjacent to the lesion had progressed. MRI showed long T1 and T2 values of the mass and the adjacent bone marrow. Bone scintigraphy and gallium scintigraphy showed prominent accumulation of radioisotopes in the scalp lesion. The mass, dura, and infiltrated bone were resected, and the surface of the brain was found to be intact. Histological examination revealed that the tumor was non-Hodgkin's lymphoma (diffuse, medium-sized, B cell lymphoma). The fact that the tumor first developed extracranially and then extended into the cranium without severe bone destruction and the fact that the dura completely protected the brain surface may indicate that the malignant lymphoma originated from the skull and showed infiltrative growth, while the dura provided a strong barrier to its intracranial extension. Thus, malignant lymphoma originating from the skull may tend to extend outside the cranium first and within the cranium subsequently, as in our case, and this may be the reason why the complaint at presentation of more than half of the reported patients is a scalp mass rather than any neurological sign.
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PMID:[A case of malignant lymphoma of the cranial vault]. 1270 23


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