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

The authors report the case of a 63-year-old woman who presented with a primary dural extranodal marginal zone lymphoma (MZL) associated with massive kappa light chain amyloidosis of the meninges. Extranodal MZL is a low-grade B-cell lymphoma that may show variable degrees of plasmacytic differentiation. Like solitary plasmacytoma of soft tissue, which can also be associated with amyloid, extranodal MZL generally responds well to local therapy and has a good prognosis. It is important to distinguish these entities from high-grade primary central nervous system (CNS) B-cell lymphomas and more aggressive and/or widespread, potentially amyloidogenic conditions such as multiple myeloma, lympho-plasmacytoid lymphoma, and chronic lymphocytic leukemia/small lymphocytic lymphoma. To the authors' knowledge this is the first reported case of dural MZL associated with massive meningeal amyloid deposition. Extranodal MZL is a rare low-grade primary CNS B-cell lymphoma that may be associated with amyloidosis. It should be considered in the differential diagnosis of CNS lymphoproliferative lesions and CNS amyloidosis.
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PMID:Dural marginal zone lymphoma with massive amyloid deposition: rare low-grade primary central nervous system B-cell lymphoma. Case report. 1183 14

Meningeal and intracerebral plasma cell granulomas are uncommon inflammatory lesions of unknown etiology. In this paper the diagnostic difficulties in two patients with meningeal plasma cell granuloma and one patient with intracerebral plasma cell granuloma are described. The first patient had an intracranial extra-axial lesion, which was first diagnosed as a meningioma. One and a half years later she underwent a second resection for recurrent tumor growth and the diagnosis of a meningeal plasma cell granuloma was made. The second patient was treated for a central nervous system B-cell lymphoma but proved to have an intracerebral plasma cell granuloma in retrospect 11 years later. In the third patient tuberculous meningitis was considered to be the most likely diagnosis because infratentorial contrast-enhanced thickened meninges (pachymeningitis) were found together with a high protein level in the cerebrospinal fluid and a positive Mantoux test. However, pathological examination of an extra-axial, cervical lesion that was operated upon revealed a meningeal plasma cell granuloma. These cases show the importance of diagnosing a meningeal or intracerebral plasma cell granuloma correctly, since it has both therapeutical and prognostic implications.
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PMID:The diagnostic difficulties of meningeal and intracerebral plasma cell granulomas--presentation of three cases. 1464 45

Patients with neurolymphomatosis show lymphoma cells within the peripheral nerves, nerve root/plexus, or cranial nerves. However, most neurolymphomatosis patients show lymphomatous infiltration not only in the peripheral nervous system (PNS), but also in the meninges, Virchow-Robin space, and brain parenchyma. Here, we report a 74-year-old woman with diffuse large B-cell lymphoma presenting with motor-sensory-autonomic polyneuropathy and multiple cranial neuropathies. A diagnosis of neurolymphomatosis was made by sural nerve biopsy. Postmortem examination indicated that lymphoma cell infiltration in the nervous system was confined to the PNS with no involvement of the central nervous system, including the meninges. This was a very rare case of B-cell neurolymphomatosis with lymphomatous infiltration confined to the PNS, suggesting specific affinity of the lymphoma cells for the PNS in this patient.
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PMID:B-cell neurolymphomatosis confined to the peripheral nervous system. 1749 Jun 87

Diffuse large B-cell lymphoma of the meninges is a particularly rare form of primary CNS lymphoma. We report a case of a 63-year-old woman found to have primary meningeal lymphoma (PML) with dural and leptomeningeal involvement whom we treated with multiple cycles of intra-arterial (IA) methotrexate, intravenous (IV) etoposide phosphate, and IV cyclophosphamide after reversible osmotic blood-brain barrier disruption (BBBD). Improvement was evident on gadolinium-enhanced brain MRI one month into therapy. At 67 months post-diagnosis there is no evidence of CNS disease. After completing her therapy regimen, she remained disease-free for 34 months, when stage IV diffuse large B-cell lymphoma was discovered in her left adrenal gland and right thigh. Following six cycles of rituximab and CHOP treatment, she is presently in complete remission. IA methotrexate and reversible osmotic BBBD without radiation therapy may be an effective therapy for treating PML.
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PMID:Primary meningeal CNS lymphoma treated with intra-arterial chemotherapy and blood-brain barrier disruption. 1875 13

The current World Health Organization (WHO) classification of central nervous system tumors lists meningeal hemangiopericytomas (HPC) and meningeal solitary fibrous tumors (SFT) as separate entities. On the contrary, SFT and HPC of soft tissues are regarded in the WHO soft tissue fascicle as features of the same entity. The clinical data, histology, and immunohistochemistry of 18 cases of meningeal HPC and 12 cases of peripheral soft tissue HPC-SFT were compared. Both intracranial and soft tissue lesions had significant similarities that included staghorn vasculature, necrotic areas, cytologic atypia, and positivities for CD99, collagen IV, and reticulin. Nevertheless, intracranial tumors were more cellular than HPC-SFT of soft tissues and had fewer collagen bands. Meningeal HPC in addition had more mitoses, higher Ki67 index, stained less intensely for CD34 and B-cell lymphoma 2 (BCL2) than HPC-SFT of soft tissues. Meningeal HPCs recurred in 13 out of 14 cases (92.9%). One of the patients died in the postoperative period for a recurrent lesion 5 years after the diagnosis, and another patient developed an extracranial metastasis 13 years after surgery. None of the six cases of HPC-SFT of soft tissues available for follow-up recurred. Both meningeal and soft tissue tumors appear to represent different features of the same entity. A more aggressive phenotype of the tumor together with incomplete surgical resection of intracranial lesions might explain the noticeable clinical difference between HPC of the meninges and HPC-SFT of soft tissues.
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PMID:Meningeal hemangiopericytomas and hemangiopericytoma/solitary fibrous tumors of extracranial soft tissues: a comparison. 2016 66

Primary brain lymphoma is a rare variant of extranodal non-Hodgkin's B-cell lymphoma. In >90% of cases, this is diffuse large B-cell lymphoma with CD20 expression and is confined to the brain, meninges, spinal cord, and eyes. It accounts for fewer than 7% of primary brain tumors. Its incidence has been rising in recent years in immunocompetent patients in their fifth and sixth decades. The rate of relapse after initial therapy based on high-dose methotrexate and/or total brain irradiation is high. There is no consensus for treating relapse, which ranges from retreatment with high doses of methotrexate, polychemotherapy, high doses of chemotherapy backed up by autologous stem-cell transplant to intrathecal chemotherapy, with widely differing results. Given the lack of consensus and poor results, new therapy options have appeared, including immunotherapy with rituximab. At a systemic level, alongside chemotherapy, its results are very modest and limited due to the low concentration it reaches in cerebrospinal fluid (CSF). However, its intrathecal and intraventricular use, though only isolated cases have been reported, has provided promising results.
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PMID:Ocular relapse of primary brain lymphoma in immunocompetent patient, treated with intrathecal rituximab. 2094 85

Streptococcus pneumoniae usually produces infection of the respiratory tract, inner ear or meninges. Unusual sites of infection have rarely been reported among HIV-1 seropositive patients. We report a case of post auricular subcutaneous abscess caused by Streptococcus pneumoniae in a Human Immunodeficiency Virus (HIV) infected child who also had B cell lymphoma. This case is uncommon as there was no other documented primary focus of pneumococcal infection or a preceding history of bacteraemia or respiratory infection.
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PMID:Unusual presentation of Streptococcus pneumoniae in human immunodeficiency virus infection. 2447 84

Background. Primary spinal dural lymphomas (PSDL) are tumors with characteristic histopathology of a lymphoma, which are completely in the spinal epidural space without any other systemic involvement. Extranodal primary lymphoma involving nervous system prefers thalamus/basal ganglia, periventricular region, cerebellum, eyes, meninges/dura, and cranial nerves or spinal cord. Rare spinal localization with acute spinal cord compression is worth attention. Case Presentation. A 48-year-old male presented with a several-month-long history of upper back pain. Lately, he had numbness and weakness at both lower extremities and was unable to walk for one week. A spinal MRI showed a thoracic lesion with cord compression at T2-T4 levels. The patient underwent surgical decompression, with his final histopathology showing diffuse large B-cell lymphoma. Systemic work-up was negative for nodal disease. Following surgery, he received radiotherapy combined with chemotherapy. He experienced a good outcome after four years. Conclusion. The upper thoracic cord is a rare location for primary spinal lesions/metastases, both of which prefer the lower thoracic and upper lumbar regions. In cases of progressive paraparesis, there should be immediate surgical intervention in the case of denovo disease, followed by combined radiotherapy and chemotherapy procedures.
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PMID:Primary Dural Spinal Lymphoma Presentation of a Rare Spinal Tumor Case. 2619 84

Primary non-Hodgkin's lymphoma (NHL) of the cranium with extra- and intracranial extension without systemic or skeletal manifestation in a non-immunocompromised patient is extremely rare. These lesions are most of the time misdiagnosed because they mimic other conditions like meningioma. Here, we report a case presented with huge bulky scalp mass which on magnetic resonance imaging (MRI) brain showed involvement of scalp, cranial vault, meninges, and the brain parenchyma, mimicking a meningioma. After gross total resection, biopsy and CD marker study revealed primary non-Hodgkin's diffuse large B-cell lymphoma (DLBCL). Malignant NHL should be considered in differential diagnosis of bulky scalp mass lesion.
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PMID:Primary non-Hodgkin's lymphoma of the skull with extra and intracranial extension presenting with bulky scalp mass lesion. 2769 53

Cranial nerve palsy could be one of the presenting features of underlying benign or malignant tumors of the head and neck. The tumor can involve the cranial nerves by local compression, direct infiltration or by paraneoplastic process. Cranial nerve involvement depends on the anatomical course of the cranial nerve and the site of the tumor. Patients may present with single or multiple cranial nerve palsies. Multiple cranial nerve involvement could be sequential or discrete, unilateral or bilateral, painless or painful. The presentation could be acute, subacute or recurrent. Anatomic localization is the first step in the evaluation of these patients. The lesion could be in the brain stem, meninges, base of skull, extracranial or systemic disease itself. We present 3 cases of underlying neoplasms presenting as cranial nerve palsies: a case of glomus tumor presenting as cochlear, glossopharyngeal, vagus and hypoglossal nerve palsies, clivus tumor presenting as abducens nerve palsy, and diffuse large B-cell lymphoma presenting as oculomotor, trochlear, trigeminal and abducens nerve palsies due to paraneoplastic involvement. History and physical examination, imaging, autoantibodies and biopsy if feasible are useful for the diagnosis. Management outcomes depend on the treatment of the underlying tumor.
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PMID:Tumors Presenting as Multiple Cranial Nerve Palsies. 2855 21


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