scholarly journals IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case

2021 ◽  
Vol 2 (23) ◽  
Author(s):  
Haggai Suisa ◽  
Jean Francois Soustiel ◽  
Yuval Grober
Keyword(s):  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Histopathological Analysis ◽  
Illustrative Case ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Total Body ◽  
Skull Base Meningiomas

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.

scholarly journals Immunoglobulin G4-related hypertrophic pachymeningitis

2019 ◽  
Vol 6 (4) ◽  
pp. e568 ◽  
Author(s):  
Michaël Levraut ◽  
Mikaël Cohen ◽  
Saskia Bresch ◽  
Caroline Giordana ◽  
Fanny Burel-Vandenbos ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Case Reports ◽  
Treatment Algorithm ◽  
Elevated Serum ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Meningeal Involvement ◽  
Serum Igg4

ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

scholarly journals Immunoglobulin G4 related disease: an overview

2021 ◽  
Vol 15 (2) ◽  
pp. 44-51
Author(s):  
Saika Farook ◽  
Abdullah Ahmed Solaiman ◽  
Md Shariful Alam Jilani
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Tubulointerstitial Nephritis ◽  
The Body ◽  
Histopathological Analysis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
First Time ◽  
Gland Enlargement

Immunoglobulin G4 related disease (IgG4-RD) is a recently perceived fibroinflammatory condition, identified as a systemic illness for the first time in the early 2000. It can involve virtually every organ of the body, commonly presenting as lymphadenopathy, retroperitoneal fibrosis, autoimmune pancreatitis, tubulointerstitial nephritis, parotid or lacrimal gland enlargement. The diagnosis is confirmed by histopathological analysis and is often, but not always accompanied by an increased level of serum IgG4 concentration. In fact, the name addressing this autoimmune fibroinflammatory condition may be considered a misnomer, as the role of the non-inflammatory immunoglobulin IgG4 in the immune mechanism of IgG4-RD remains to be elucidated. Ibrahim Med. Coll. J. 2021; 15(2): 44-51

Motion-related vascular abnormalities at the craniocervical junction: illustrative case series and literature review

2015 ◽  
Vol 38 (4) ◽  
pp. E6 ◽  
Author(s):  
Vijay M. Ravindra ◽  
Jayson A. Neil ◽  
Marcus D. Mazur ◽  
Min S. Park ◽  
William T. Couldwell ◽  
...  
Keyword(s):  
Vertebral Artery ◽  
Case Series ◽  
Vascular Anomaly ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Dissecting Aneurysm ◽  
Vascular Pathology ◽  
Illustrative Case ◽  
Vascular Compression ◽  
Elective Resection

The craniocervical junction (CCJ) functions within a complicated regional anatomy necessary to protect and support vital neurovascular structures. In select instances, vascular pathology can be attributed to this complicated interplay of motion and structure found within this narrow space. The authors report 3 cases of complex vascular pathology related to motion at the CCJ and detail the management of these cases. Two cases involved posterior circulation vascular compression syndromes, and one case involved a vascular anomaly and its relation to aneurysm formation and rupture. The patient in Case 1 was a 66-year-old man with a history of syncopal episodes resulting from the bilateral vertebral artery becoming occluded when he rotated his head. Successful microsurgical decompression at the skull base resulted in patent bilateral vertebral artery V3 segments upon head movement in all directions. The patient in Case 2 was a 53-year-old woman who underwent elective resection of a right temporal meningioma and who experienced postoperative drowsiness, dysphagia, and mild right-arm ataxia. Subsequent MRI demonstrated bilateral posterior inferior cerebel-lar artery (PICA) strokes. Cerebral angiography showed a single PICA, of extradural origin, supplying both cerebellar hemispheres. The PICA exhibited dynamic extradural compression when the patient rotated her head; the bilateral PICA strokes were due to head rotation during surgical positioning. In Case 3, a 37-year-old woman found unconscious in her home had diffuse subarachnoid hemorrhage and evidence of a right PICA aneurysm. A right far-lateral craniectomy was performed for aneurysm clipping, and she was found to have a dissecting aneurysm with an associated PICA originating extradurally. There was a shearing phenomenon of the extradural PICA along the dura of the foramen magnum, and this microtraumatic stress imposed on the vessel resulted in a dissecting aneurysm. This series of complex and unusual cases highlights the authors’ understanding of vascular pathology of the CCJ and its management.

scholarly journals IgG4 Related Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Sevda Akyol ◽  
Ozlem Saraydaroglu ◽  
Omer Afsin Ozmen
Keyword(s):  
Plasma Cells ◽  
Clinical Laboratory ◽  
Review Of The Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Igg4 Level

Abstract Objectives: Immunoglobulin G4–related disease is characterized by increased serum IgG4 level, enlargement in the relevant organs and histopathologically intense storiform fibrosis, lymphoplasmacytic infiltration rich in IgG4 positive plasma cells, and obliterative phlebitis.Methods and Results: In this report, a patient who underwent a laryngeal biopsy with a pre-diagnosis of malignancy, but had findings consistent with immunoglobulin G4–related disease in the biopsy sample, is described.Conclusion: Immunoglobulin G4–related disease can be seen in very rare localizations. It should be kept in mind in differential diagnosis when tissues especially containing inflammation rich in plasma cells are encountered. Clinical, laboratory and pathological correlation is extremely important in the diagnosis of an IgG4-related disease.

Low-dose ‘boom-boom’ radiotherapy for ocular lymphoma arising from IgG4-related ophthalmic disease: Case report and literature review

2021 ◽  
pp. 112067212110183
Author(s):  
Katherine Meng ◽  
Mei Chin Lim ◽  
Michelle Li Mei Poon ◽  
Gangadhara Sundar ◽  
Balamurugan Vellayappan
Keyword(s):  
Low Dose ◽  
Elevated Serum ◽  
Infraorbital Nerve ◽  
Immunoglobulin Light Chain ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Disease Case ◽  
Serum Igg4 ◽  
Ophthalmic Disease

Ocular adnexal extranodal marginal zone lymphoma (OA-EMZL) and immunoglobulin G4-related ophthalmic disease (IgG4-ROD) may exist on a continuum. Presence of immunoglobulin light-chain restriction and clonal gene rearrangement suggests presence of lymphoma; whereas bilateral, infraorbital nerve and systemic involvement accompanied by elevated serum IgG4 levels may indicate synchronous IgG4-ROD. Although steroids have been the mainstay for the treatment of IgG4-ROD, radiotherapy (RT) has been used occasionally. The reported RT doses range between 24 and 30 Gy, which can result in acute and late toxicities. A low-dose regimen of four Gy has not been previously described. We describe a patient with bilateral OA-EMZL arising from IgG4-ROD successfully treated with low dose ‘boom-boom’ radiotherapy. In addition, we review the literature for the association between these two conditions and the role of RT in their management.

scholarly journals IgG4 Related Disease of Epididymis, Mimicking Testicular Malignancy – A Rare Entity

2019 ◽  
pp. 1-4
Author(s):  
LEELA KRISHNA ◽  
SRIRAM KRISHNAMOORTHY ◽  
HARIHARASUDHAN SEKAR ◽  
SUSRUTHAN MURALI ◽  
RAJENDIRAN SWAMINATHAN ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Rare Entity ◽  
Left Testis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Related Disease ◽  
Left Testicle ◽  
Serum Igg4 ◽  
Spindle Cell Proliferation

Immunoglobulin G4 related disease (IgG4-RD) is a systemic fibro inflammatory condition that usually presents with multiorgan involvement. We present a rare case of 54 year old male with an isolated IgG4-RD of epididymis. The patient presented with a progressive swelling of the left testicle. A clinical diagnosis of tuberculosis was made. Ultrasound scrotum showed a relatively hetero-echoic mass lesion involving the left epididymis in close proximity to the left testis. There was a focal spindle cell proliferation and an increase in number of plasma cells and keloid like collagen. Immunohistochemistry was positive for vimentin and IgG4 and negative for CD34. Serum level of IgG4 was elevated (165 mg per dL). Computed tomography of abdomen and thorax did not show any systemic involvement. HE was posted for excision of the epididymal mass. Intraoperatively, the mass was found to be densely adherent to left testicle and inseparable from it, necessitating left total orchiectomy. Histopathology and immunohistochemistry with elevated serum IgG4 levels confirmed the diagnosis of IgG4-RD of the epididymis. To the best of our knowledge, this condition is an extremely rare entity, with only very few cases of isolated IgG4-RD of epididymis reported in medical literature, with no other systemic manifestations.

scholarly journals Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Hounsfield Unit ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
Orbital Lymphoma ◽  
Serum Igg Levels ◽  
Igg Level ◽  
Ct Features ◽  
Higher Sensitivity

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

scholarly journals IgG4-related gastric disease with plasma cell-rich obliterative arteritis accompanied by early-stage gastric cancer: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Masayoshi Obatake ◽  
Koichi Sato ◽  
Shigehiko Yagi ◽  
Hiromi Ohtani ◽  
Katsumi Kito
Keyword(s):  
Gastric Cancer ◽  
Plasma Cell ◽  
Early Stage ◽  
Vascular Lesions ◽  
Gastric Disease ◽  
Inflammatory Disorder ◽  
Obliterative Phlebitis ◽  
Regional Lymph Node Dissection ◽  
Serum Igg4 ◽  
Storiform Fibrosis

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder that can involve multiple organs. It is characterized by IgG4-positive plasma cell-rich storiform fibrosis and obliterative phlebitis associated with a high serum IgG4 level. There are few reports of gastric IgG4-RD, especially those detected prior to systemic or other organ involvement. Case presentation: A 70-year-old man was diagnosed with type 0–IIc gastric cancer at the anterior wall of the gastric corpus by upper gastrointestinal endoscopy. In addition, a submucosal tumor (SMT) 7 mm in diameter was found at the greater curvature of the angulus. Laparoscopic distal gastrectomy with regional lymph node dissection was performed. Pathology revealed a poorly differentiated adenocarcinoma in the type 0–IIc lesion and storiform fibrosis with infiltration of a large number of IgG4-positive plasma cells in the SMT. Postoperative laboratory testing showed elevation of serum IgG4 levels; thus, we diagnosed the SMT as IgG4-RD. Intriguingly, the gastric IgG4-RD lesion demonstrated IgG4-positive plasma cell-rich arteritis as well as typical obstructive phlebitis. The patient has been followed for 2 years after surgery without recurrence of cancer, but skin lesions of IgG4-RD have appeared. Conclusion We report a rare case of IgG4-RD presenting as a gastric SMT, accompanied by early-stage gastric cancer. Our case may support a newly proposed relationship between IgG4-RD and malignancies. The gastric IgG4-RD lesion showed arteritis as well as obliterative phlebitis, potentially providing novel insight into IgG4-related vascular lesions.

scholarly journals Immunoglobulin G4-related masses surrounding coronary arteries: a case report

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Takuya Nakamura ◽  
Yutaka Goryo ◽  
Takuya Isojima ◽  
Hiroyuki Kawata
Keyword(s):  
Coronary Artery ◽  
Coronary Angiography ◽  
Cardiovascular System ◽  
Coronary Arteries ◽  
Elevated Serum ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Coronary Arteritis ◽  
Serum Igg4 ◽  
Mass Formation

Abstract Background Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries. Case summary A 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images. Discussion IgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.

scholarly journals Far Lateral Craniotomy for Resection of Foramen Magnum Meningioma

2019 ◽  
Vol 80 (S 04) ◽  
pp. S355-S357
Author(s):  
Robert T. Wicks ◽  
Xiaochun Zhao ◽  
Celene B. Mulholland ◽  
Peter Nakaji
Keyword(s):  
Cranial Nerves ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Occipital Condyle ◽  
Formidable Challenge ◽  
Close Proximity ◽  
Vertebrobasilar Circulation ◽  
Far Lateral ◽  
Lower Cranial Nerves ◽  
Gain Access

Abstract Objective Foramen magnum meningiomas present a formidable challenge to resection due to frequent involvement of the lower cranial nerves and vertebrobasilar circulation. The video shows the use of a far lateral craniotomy to resect a foramen magnum meningioma. Design, Setting, and Participant A 49-year-old woman presented with neck pain and was found to have a large foramen magnum meningioma (Fig. 1A, B). Drilling of the posterior occipital condyle was required to gain access to the lateral aspect of the brain stem. The amount of occipital condyle resection varies by patient and pathology. Outcome/Result Maximal total resection of the tumor was achieved (Fig. 1B, C), and the patient was discharged on postoperative day 4 with no neurologic deficits. The technique for tumor microdissection (Fig. 2) is shown in the video. Conclusion Given the close proximity of foramen magnum meningiomas to vital structures at the craniocervical junction, surgical resection with careful microdissection and preservation of the overlying dura to prevent postoperative pseudomeningocele is necessary to successfully manage this pathology in those patients who are surgical candidates.The link to the video can be found at: https://youtu.be/Mds9N1x2zE0.

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