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IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review

IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a...

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Autores principales: Liu, Xiaohai, Wang, Renzhi, Li, Mingchu, Chen, Ge
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8120283/
https://www.ncbi.nlm.nih.gov/pubmed/33995286
http://dx.doi.org/10.3389/fendo.2021.666791
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author Liu, Xiaohai
Wang, Renzhi
Li, Mingchu
Chen, Ge
author_facet Liu, Xiaohai
Wang, Renzhi
Li, Mingchu
Chen, Ge
author_sort Liu, Xiaohai
collection PubMed
description IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days’ duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient’s symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis.
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spelling pubmed-81202832021-05-15 IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review Liu, Xiaohai Wang, Renzhi Li, Mingchu Chen, Ge Front Endocrinol (Lausanne) Endocrinology IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days’ duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient’s symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis. Frontiers Media S.A. 2021-04-30 /pmc/articles/PMC8120283/ /pubmed/33995286 http://dx.doi.org/10.3389/fendo.2021.666791 Text en Copyright © 2021 Liu, Wang, Li and Chen https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Liu, Xiaohai
Wang, Renzhi
Li, Mingchu
Chen, Ge
IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title_full IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title_fullStr IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title_full_unstemmed IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title_short IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review
title_sort igg4-related inflammatory pseudotumor involving the clivus: a case report and literature review
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8120283/
https://www.ncbi.nlm.nih.gov/pubmed/33995286
http://dx.doi.org/10.3389/fendo.2021.666791
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