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(A) Low magnifi cation shows a dense lymphocytic infi ltrate with vague nodularity; there is sclerosis in the background. (B) High magnifi cation shows that the infi ltrate is composed predominantly of small round lymphocytes with clear cytoplasm admixed with scattered small mature plasma cells. (C) High magnifi cation shows a mixed infi ltrate with small to intermediate and rare large size lymphocytes admixed with numerous plasma cells. (D) Immunohistochemistry for the B-cell marker CD20 highlights small to intermediate size lymphocytes. Many negative cells are likely plasma cells. (E) Immunohistochemistry for immunoglobulin kappa light chain demonstrates that most plasma cells are positive (monotypic kappa). (F) Immunohistochemistry for immunoglobulin G4 (IgG4) demonstrates that scattered plasma cells are positive.
Contexts in source publication
Context 1
... e patient is alive, free of disease 12 months from initial diagnosis. All three patients had normal blood counts, renal and liver function, lactate dehydrogenase, β 2 -microglobulin and serum protein electrophoresis, and were seronegative for human immunodefi ciency virus (HIV) and hepatitis B and C. All three cases showed sclerotic dura marrow with a diff use lymphoid infi ltrate [ Figure 1(A)]. Th e infi ltrate was composed of small lymphocytes with round to irregular nuclei and an occasional intermediate size lympho- cyte with vesicular chromatin. ...
Context 2
... e infi ltrate was composed of small lymphocytes with round to irregular nuclei and an occasional intermediate size lympho- cyte with vesicular chromatin. Some lymphocytes displayed clear cytoplasm, with a monocytoid appearance [ Figure 1(B)]. No mitotic fi gures or necrosis was detected. ...
Context 3
... mitotic fi gures or necrosis was detected. In case 1, a signifi cant number of lymphocytes showed clear cyto- plasm [ Figure 1(B)] and no plasmacytic diff erentiation was noted. In cases 2 and 3, the infi ltrate was more polymorphic and admixed with numerous small, mature plasma cells [ Figure 1(C)]. ...
Context 4
... case 1, a signifi cant number of lymphocytes showed clear cyto- plasm [ Figure 1(B)] and no plasmacytic diff erentiation was noted. In cases 2 and 3, the infi ltrate was more polymorphic and admixed with numerous small, mature plasma cells [ Figure 1(C)]. Immunohistochemical studies showed that most cells in the infi ltrate were positive for CD20 [Figure 1 ...
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... Regarding the role of infectious agents in the development of the disease, none were identified [2]. Due to a strong predominance in the female population, one could hypothesize a hormonal role in its pathophysiology [8]. ...
... In MALT lymphomas of other sites (not associated with infectious causes), treatment involving radiotherapy or surgical resection offers good results [9]. In a systematic review by Beltran et al. [8] that included 91 patients with primary MALT lymphomas of the dura, 70% received surgical treatment, 73% radiotherapy and 37% chemotherapy. Chemotherapy included intrathecal methotrexate, CHOP scheme, among others. ...
Primary MALT lymphoma arising at the dura is a rare circumstance with no categorical therapeutic plan in literature. There are few reports available with different treatment courses. Here, we report two cases with a long-term follow-up after the same pattern of management and review the literature.
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Primary presentation of intradural non-Hodgkin lymphoma (PDL) is rare. Most published cases of PDL are single-patient reports. This lesion occurs more often in middle-aged women, in contrast to brain parenchyma primary central nervous system lymphoma (PCNSL), which has a slight male predilection. In contrast with parenchymal PCNSL, there has been no clear association between acquired and congenital immunosuppression and the development of PDL. The pathogenesis of PDL is not well understood because the dura is devoid of any lymphoid tissue. One hypothesis is that a benign inflammatory condition of the dura could attract polyclonal lymphocytes from which a monoclonal lymphoma could arise. B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) have been recognized as an important pathologic subtype. A PDL is more indolent and has a better prognosis than parenchymal PCNSL or systemic lymphoma with CNS metastasis. However, further studies with longer clinical follow up are necessary to assess the final outcome in these patients. In general, patients with marginal zone lymphoma have a favorable outcome, with a 5-year overall survival rate greater than 86%, without significant differences between gastrointestinal and nongastrointestinal and between localized and disseminated disease.
... The overwhelming majority of PDLs are marginal zone lymphomas similar to the mucosa-associated lymphoid tissue (MALT) lymphoma commonly found in gastric mucosa (1,2,5,6,(9)(10)(11)(12)(13)(14). MALT lymphoma of the gastric mucosa is most commonly associated with Helicobacter pylori infection but it has also been linked to other infections (15)(16)(17)(18). ...
Central nervous system (CNS) involvement in sarcoidosis is rare and typically occurs in 5-10% of patients. Neurological symptoms in a patient with known sarcoidosis can be attributed to neurosarcoidosis without thorough evaluation. Primary Dural Lymphoma (PDL) is an extremely rare form of non-Hodgkin lymphoma. Although PDL is technically a subtype of primary CNS lymphoma, the two entities vary markedly in their histological grade, clinical course, prognosis and treatment. The most common dural- based lesion found on CNS imaging is meningioma. It shares many imaging, clinical and epidemiologic features of PDL which often leads to misdiagnosis of PDL as meningioma. We present a case where a PDL was diagnosed after CNS symptoms failed to resolve after steroid therapy for presumed neurosarcoidosis.
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... plasma cells are suspected to arise from IgG4 sclerosing disease. [13][14][15] However, a recent report found a high percentage (39%) of primary cutaneous MZLs to have IgG4 expression, while from 120 noncutaneous MALT lymphomas, only one case, located in the ocular adnexae, expressed IgG4. 16 None of the reported cases had evidence of an associated IgG4related disease. In our case, we identified more than 50 plasma cells per high power field expressing IgG4, raising the suspicion of an underlying IgG4 sclerosing disease. ...
: Extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue usually originates from cutaneous or mucosal surfaces. A rare site of involvement is the subcutaneous tissue of any location. Here, we describe a 58-year-old man who presented with bilateral extranodal MZL of mucosa-associated lymphoid tissue from ocular adnexae that involved subcutaneous tissue and subsequently extended to multiple anatomical locations in the head and neck, upper back, and arm. The neoplastic cells expressed B-cell markers, and the plasma cells expressed IgG4. The unusual pattern of infiltration of this extranodal MZL and the possible significance of IgG4 expression in this case are discussed.
Marginal zone lymphoma (MZL) is the most common indolent lymphoma primarily arising in the central nervous system (CNS). To date, 207 cases of primary CNS MZL (PCNSMZL) were published, mostly as single case reports or small case series. It most commonly presents as extra‐axial dural‐based masses, more frequently in middle‐aged women, displaying an insidious onset, with a long history of symptoms preceding the diagnosis. PCNSMZL can be radiographically mistaken for meningioma. PCNSMZL consists of CD20 ⁺ , CD3 ⁻ small B lymphocytes with varying degrees of plasmacytic differentiation and low proliferation index. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality. Other recurrent genetic abnormalities involve TNFAIP3 and NOTCH2. Ethiopathogenesis was poorly investigated. Due to its rarity, standard of care remains to be defined; it exhibits an excellent prognosis after varied treatments, such as surgery, radiotherapy, chemotherapy or their combinations. Nevertheless, each treatment should be considered after an accurate analysis of overtreatment risk. Short follow‐up is a major limitation in reported PCNSMZL cases, which restrains our knowledge on long‐term results and iatrogenic sequels. This review was focussed on presentation, differential diagnoses, pathological findings, treatment options and clinical outcomes of PCNSMZL; recommendations for best clinical practice are provided.
Mucosa-associated lymphoid tissue (MALT) was first described as low-grade lymphoma associated with the stomach mucosa. Although the stomach and ocular adnexa are the most common localizations of MALT lymphoma, it has also been described in many other organs, including the head and neck, lungs, thyroid, breast, bladder, saliva glands, conjunctiva, and tear glands. MALT lymphoma originating from the dura is rare. The case is here presented of an 83-year-old female operated on with an initial diagnosis of acute subdural hematoma. In the histopathological examination, there was seen to be lymphoplasmacytic infiltration of the dura and a lymphomeningothelial lesion. Immunohistochemically, low-grade MALT lymphoma showing B-cell phenotype was considered. This is the first reported case of lymphomeningothelial lesion in MALT lymphoma originating from the dura.
Objectives
Dural based Marginal Zone MALT-type B-Cell Lymphoma (MZBCL) is an intracranial tumor that can mimicking meningioma both from a clinical and a radiological point of view. A standard treatment protocol is still lacking. Aim of the present work is to provide an update of the present literature regarding this rare neoplasia
Patients and methods
We report the case of a patient with a dural-based lesion mimicking a meningioma of the tentorium. After surgical treatment, the diagnosis was of MZBCL. A literature review is performed to highlight the typical characteristics of this rare intracranial lesion and to define the best therapeutic approach.
Results
Literature review included 38 articles describing 126 cases of intracranial dural-based MZBCL. No clinical trial has been found. Clinical and histopathological features are properly collected to provide a guide for future cases. Different treatment options have been attempted. Combination of surgery with adjuvant radiation therapy is the most used option.
Conclusions
MZBCL should be considered in differential diagnosis for dural-based intracranial lesion. Surgery followed by radiation therapy is the most reported treatment. As a consequence of the rarity of this disease, of its indolent progression and of the lack of adequate follow-up, it is not possible to define it is the best treatment option.
Background
Primary extranodal marginal zone lymphoma (MZL) of the dura is a rare neoplastic entity in the central nervous system (CNS).
Methods
We used literature searches to identify previously reported cases of primary dural MZL. We also reviewed clinical, pathologic, and radiographic data of an adult patient with concurrent dural MZL and chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL).
Results
We identified 104 cases of dural MZL in the literature. None of them presented concurrently with another type of non-Hodgkin lymphoma. This is the first report of composite lymphoma consisting of dural MZL and CLL/SLL in the bone marrow and lymph nodes.
Conclusion
Primary dural MZL is a rare, indolent low-grade CNS lymphoma, with a relatively good prognosis. Its treatment is multidisciplinary and often requires surgical intervention due to brain compression, along with low to moderate doses of radiotherapy and/or systemic chemotherapy.
Extranodal marginal zone lymphoma of the central nervous system (CNS EMZBL) is a rare disease. We present a review of the literature and describe its presentation, differential diagnosis, treatment options, and outcomes. Systematic search of PubMed, Medline, and Embase databases via the Ovid engine for primary articles and case reports yielded 37 unduplicated peer-reviewed articles of CNS EMZBL. We identified 69 cases in these articles and 1 unreported case at our institution, which were included for this review's analysis. Median age at diagnosis was 55 years (range, 18-78 years), with a female preponderance of 77% (n = 54). Most common presenting symptoms were headache in 43% (n = 30), seizures in 31% (n = 22), and visual defects in 27% (n = 19). The most common treatment modalities were localized therapies, which were provided to 67% (n = 47) of cases. These included radiotherapy in 27% (n = 19), radiotherapy with surgery in 24% (n = 17), and surgery alone in 16% (n = 11). Ninety percent (n = 63) of patients had a median follow-up of 23 months. Complete remission was experienced by 77% (n = 49) patients, and 22% (n = 14) were alive with disease. Three patients had evidence of relapse, and one patient died. CNS EMZBL is an indolent, low-grade, radiosensitive lymphoma with good treatment outcomes and prognosis. It is an important differential to consider in extra-axial dural-based masses. Individualized management plans, with preference given to localized treatment options, should be considered after factoring in the site and extent of disease, its resectability, and the expected adverse effects of systemic therapy.
