Primary IgG4‐producing extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma) in the cavernous sinus: A mimicker of IgG4‐related disease/hypertrophic pachymeningitis

Yeh, Chao‐Hung; Tsui, Yu-Kun; Liu, Hongxiang; Chuang, Shih‐Sung

doi:10.1111/pin.13070

Cited by 4 publications

(10 citation statements)

References 5 publications

(8 reference statements)

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“…Depending on the location of the disease, isolated lesions often mimicked a diverse spectrum of skull/skull base pathologies including lymphoma, sarcoma, nasopharyngeal carcinoma, neurosarcoidosis, granulomatosis with polyangiitis, sarcoma, giant cell arteritis, Langerhans cell histiocytosis, and benign skull base tumors. [6,12,13,32,38] Radiologically, IgG4-related mass lesions, as in our case, present as homogenously enhanced lesions, which are isointense to hypointense on T1-weighted and T2-weighted images. [6,12,13,32,38,40] ree histopathological findings characterize the disease in the affected organ: (1) e presence of a storiform pattern of sclerosis; (2) a dense lymphoplasmacytic infiltrate; and (3) an increased proportion of IgG4-positive cells with respect to IgG-positive cells according to immunohistochemical evidence.…”

Section: Discussionmentioning

confidence: 51%

“…[6,12,13,32,38] Radiologically, IgG4-related mass lesions, as in our case, present as homogenously enhanced lesions, which are isointense to hypointense on T1-weighted and T2-weighted images. [6,12,13,32,38,40] ree histopathological findings characterize the disease in the affected organ: (1) e presence of a storiform pattern of sclerosis; (2) a dense lymphoplasmacytic infiltrate; and (3) an increased proportion of IgG4-positive cells with respect to IgG-positive cells according to immunohistochemical evidence. Although elevated concentrations of IgG4 in tissue and serum are helpful in diagnosing IgG4-RD, neither one is a specific diagnostic marker.…”

Section: Discussionmentioning

confidence: 51%

“…IgG4-RD cases are currently more frequently reported in Asian countries. [ 6 , 12 , 18 , 31 , 34 - 38 ] In our case, the patient was a 54-year-old female with IgG4-RD invasion of the right cavernous sinus and orbit.…”

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

“…Radiologically, IgG4-related mass lesions, as in our case, present as homogenously enhanced lesions, which are isointense to hypointense on T1-weighted and T2-weighted images. [ 6 , 12 , 13 , 32 , 38 , 40 ]…”

Section: Discussionmentioning

confidence: 99%

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Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Mayeku

Deisch

López-González

2021

Surgical Neurology International

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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Section: Discussionmentioning

confidence: 51%

Section: Discussionmentioning

confidence: 51%

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Mayeku

Deisch

López-González

2021

Surgical Neurology International

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Clinical characteristics and outcomes of IgG4‐positive marginal zone lymphoma: Systematic scoping review

Nishimura

Wien

Nishimura

et al. 2022

Pathology International

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Immunoglobulin G4 (IgG4)‐positive marginal zone lymphoma (MZL) is rare and undefined. It is unclear whether IgG4‐positive MZLs have as favorable an outcome as MZLs in general. Also, correlation with IgG4‐related disease (IgG4‐RD) and IgG4‐positive MZLs is unknown. Following the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses extension for scoping reviews, we searched MEDLINE and EMBASE for all peer‐reviewed articles using keywords including“IgG4” and “marginal zone lymphoma” from their inception to February 20, 2022. Twenty‐two articles, including six observational studies and 24 cases from 16 case reports and case series, were included. Only one study had a comparative group, and the other five were exploratory observational studies. IgG4‐positive MZLs commonly occurred in males (83.3%). It primarily involved ocular adnexa (41.7%) and skin (29.2%). Only 29.2% had concurrent IgG4‐RD, and no expiration was noted. While most cases were treated with excision, resection, or clinical observation, 21.7% received rituximab‐cyclophosphamide, doxorubicin, vincristine, and prednisone as a first‐line treatment. This systematic review summarizes the current understanding of the characteristics of IgG4‐positive MZLs. While there seems to be IgG4‐RD‐related and de novo IgG4‐positive MZLs, future research needs to clearly define MZL with polyclonal IgG4‐positive cells and IgG4‐producing lymphoma. Further studies are critical to clarifying long‐term prognosis and optimal surveillance planning.

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Clinical relevance of molecular aspects in extranodal marginal zone lymphoma: a critical appraisal

Raderer

Kiesewetter

2023

Ther Adv Med Oncol

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Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is among the more common types of lymphoma accounting for up to 8% of newly diagnosed lymphoma cases. As opposed to other B-cell lymphomas, however, no predominant genetic hallmark has been defined in MALT lymphoma, but different localizations appear to be affected by different, sometimes distinct changes. Nonetheless, a high proportion of these genetic changes reported in MALT lymphomas dysregulate the pathways leading to NF-kB activation. t(11;18)(q21;q21)/BIRC3::MALT1 appears to be MALT lymphoma specific and is found in 24% of gastric and 40% of pulmonary MALT lymphomas. The translocation is associated with more disseminated disease in gastric MALT lymphoma and is found in a large percentage of patients whose lymphoma is unresponsive to antibiotic eradication of Helicobacter pylori. In addition to t(11;18)(q21;q21), nuclear expression of BCL10 or NF-kB appears to be highly associated with lymphoma cell survival independence of H. pylori-mediated stimulations. Antibiotic eradication, however, is the recommended therapy of choice irrespective of genetic findings, and molecular analysis is not required before initiation of therapy. The influence of genetic translocations including t(11;18)(q21;q21) on systemic therapies, however, is less clearly defined. While small series have shown no influence on the outcome for treatment with the anti-CD20 antibody rituximab (R) or treatment with cladribine (2-CdA), conflicting data have been reported for alkylating agents, especially chlorambucil and the combination of R + chlorambucil. None of other genetic changes seen in MALT lymphoma to date has discernible value in routine clinical applications, but recent data suggest that changes in TNFAIP3(A20), KMTD2 and CARD11 might be associated with response to Bruton kinase inhibitors.

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Primary IgG4‐producing extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma) in the cavernous sinus: A mimicker of IgG4‐related disease/hypertrophic pachymeningitis

Cited by 4 publications

References 5 publications

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Clinical characteristics and outcomes of IgG4‐positive marginal zone lymphoma: Systematic scoping review

Clinical relevance of molecular aspects in extranodal marginal zone lymphoma: a critical appraisal

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