Immunoglobulin G4-Related Disease Masquerading an Epidural Spinal Cord Tumor

Abdulla, Ebtesam; Luther, Harleen; Shah, Tejal; Chandran, Nisha Suyien

doi:10.1055/s-0041-1722841

Cited by 2 publications

(2 citation statements)

References 6 publications

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“…The research by Ferreira et al (11) showed that plasmacytosis was first detected in biopsy of inflammatory pseudotumor, with more than 50% IgG4-positive plasma cells in the thoracic spine. We used the PubMed database to identify seven cases (2016-2020) of IgG4-RD IPT (5)(6)(7)(8)(9)(10)(11). In these cases, the mean age of the patients was 44.5 years, with the majority being male.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-Related Spinal Intramedullary Inflammatory Pseudotumor: A Case Report and Literature Review

Zhou

Liu

et al. 2022

Front. Neurol.

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Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disease that affects several organs. An inflammatory pseudotumor is a histologically proven benign tumor-like lesion that most commonly involves the lung and orbit. It is rare in the central nervous system, but rarest in the spinal canal. In this report, we present a case of IgG4-related intramedullary spinal inflammatory pseudotumor, along with a literature review. A 29-year-old male was transferred to the Department of Neurosurgery of Lanzhou University Second Hospital with progressive quadriparesis after numbness and weakness in both lower limbs for 50 days. Enhanced magnetic resonance imaging (MRI) of the spine revealed an isointense signal on T1-weighted images and a hyperintense signal on T2-weighted images from an enhanced mass located at the thoracic vertebrae region, for which a schwannoma was highly suspected. Then, a posterior median approach was adopted. The lesion was resected. The patient received further glucocorticoid after the diagnosis of an IgG4-related inflammatory pseudotumor was established, and the patient's symptoms improved, such as quadriparesis and lower limb weakness. This case highlights the importance of considering IgG4-related inflammatory pseudotumor as a differential diagnosis in patients with lesions involving the spinal intramedullary compartment and lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumor is etiologically unclear and prognostically unpredictable, and imaging may not help establish the diagnosis of IgG4-related inflammatory pseudotumor due to its resemblance to malignant tumors, and total resection might not be warranted. Glucocorticoid and surgery are usually the first-line treatments used.

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Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-Related Spinal Intramedullary Inflammatory Pseudotumor: A Case Report and Literature Review

Zhou

Liu

et al. 2022

Front. Neurol.

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“…Immunoglobulin (Ig)G4-related disease belongs to a rare compilation of conditions in rheumatology, its varied manifestation make it a diagnostic challenge among other lesions which more frequently present as mass lesions [ 1 ]. The multitude of organs that are affected in IgG4-related disease commonly includes salivary glands, pancreas, liver, lungs, heart, and kidney [ 2 , 3 ]. However, the facade of central nervous system (CNS) manifestations in IgG4-related disease makes them mystifying and is less documented [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Mass: The Conundrum in Spine

Rath¹,

Katoch²,

Agrawal³

et al. 2023

JOCR

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Introduction: Immunoglobulin (Ig)G4-related disease belongs to a rare compilation of conditions in rheumatology and may involve a multitude of organs. Amidst the central nervous system (CNS) presentation, involvement of spinal cord is rarer still. Case Report: A 50-year-old male came with chief complaints of tingling sensation over both soles since 2 months associated with lower back ache and gait disturbance in the form of spastic gait. X-rays of spine were suggestive of a growth at the level of D10–D12 compressing the spinal cord with no focal sclerotic/lytic lesions, and MRI dorsolumbar spine showed dural tail sign. The patient underwent excision of dural mass, and histopathology revealed majority of plasma cells positive for IgG4. Case 2: A 65-year-old female came with complaints of cough, shortness of breath, and fever on and off since 2 months. No history of hemoptysis, purulent sputum, weight loss. On examination: There were bilateral rhonchi in the left upper zone. MRI spine showed focal erosion with soft-tissue thickening at the right paravertebral region extending from D5-9. The patient underwent surgery (fusion D6-8 and ostectomy D7 posterior rib resection D7 right side) with the right pleural biopsy and transpendicular intracorporal biopsy D7. Histopathology was compatible with findings of IgG4 disease. Discussion: IgG4 tumors presenting in CNS itself is rare and rarer in spinal cord. Histopathological examination is central to diagnose and prognosticate the disease further as IgG4-related disease manifestations may recur without proper treatment. Keywords: IgG4, spinal mass, central nervous system, pleura.

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Immunoglobulin G4-Related Disease Masquerading an Epidural Spinal Cord Tumor

Cited by 2 publications

References 6 publications

Immunoglobulin G4-Related Spinal Intramedullary Inflammatory Pseudotumor: A Case Report and Literature Review

Immunoglobulin G4-Related Spinal Intramedullary Inflammatory Pseudotumor: A Case Report and Literature Review

Mass: The Conundrum in Spine

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