Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

Sato, Mototaro; Kodaira, Minori; Ishii, Wataru; Nakagawa, Michitaka; Kishida, Dai; Miyazaki, Daigo; Shimojima, Yasuhiro; Matsuda, Masayuki; Uehara, Takeshi; Ikeda, Shu‐ichi

doi:10.2169/internalmedicine.54.3230

Cited by 8 publications

(3 citation statements)

References 28 publications

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“…The full texts of 530 articles were read, and 116 articles were shortlisted for the final extraction, 112 of which were case reports and 4 were case series. 3,18...…”

Section: Resultsmentioning

confidence: 99%

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

et al. 2023

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Objectives: Multifocal fibrosclerosis is a rare disorder causing progressive fibrosis of multiple organ systems. Existing data on the disease show that there are multiple manifestations of the disease, with different outcomes. However, quantitative data are scarce, prompting the need for our investigation. Method: A comprehensive systematic review was performed from inception to November 16, 2022, with the restriction of English language, not including review articles. Article screening and extraction was performed independently, and shortlisted articles were assessed for bias. Analysis was performed using SPSS Version 25 (IBM® SPSS® Statistics; Chicago, IL, USA). Data were presented as frequencies and percentages, with a confidence interval of 95%. Result: This review included 134 patients, with 78 (58.2%) males. Mean age was 53.6 years and included two pediatric patients. The most common comorbidity was diabetes (9.7%). Prevalent presenting symptoms included pain (47.8%) and swelling (35.1%). A mean of 2.51 organs or anatomical sites was affected, retroperitoneum (64.2%) being most affected. The pancreas (30.0%) and digestive system (47.0%) were the organs/organ systems most affected. Patients had favorable outcomes in 79.1% of cases, 87.3% had no relapse, and 91.8% of patients survived the condition. Conclusion: The findings in this study provide a quantitative measurement of the demographics, presentations, organ manifestations, and outcomes of multifocal fibrosclerosis. We found the disease to be prevalent in males in Japan or the United States, with the abdomen and its organs being commonly involved.

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“…The full texts of 530 articles were read, and 116 articles were shortlisted for the final extraction, 112 of which were case reports and 4 were case series. 3,18...…”

Section: Resultsmentioning

confidence: 99%

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

et al. 2023

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“…In this disease, the cranial part of the dura is predominantly involved, but over the past years several case reports have been published describing involvement of the spinal dura (2, 5–10). Most of these patients with hypertrophic pachymeningitis had back pain and neurological complaints (5–7, 11).…”

Section: Discussionmentioning

confidence: 99%

First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions

et al. 2019

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IgG4-related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, including, but rarely, bone marrowinvolvement. Here we present a case of a 76-year-old male with IgG4-related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storiform fibrosis, and increased IgG4 positive plasma cells. Treatment with corticosteroids significantly improved wellbeing and resolved lesion size on MRI.

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“…A previous report mentioned that some patients with IgG4‐related disease and mediastinal tumors had pleuritic chest pain, which resulted in dyspnea and chylothorax . Therefore, even if a patient does not report symptoms, the manifestation must not be dismissed, and a “watch‐and‐wait” strategy should be implemented to eliminate or reduce the risks to the patient.…”

Section: Discussionmentioning

confidence: 99%

Video‐assisted thoracoscopic surgery can help enable the complete resection of a mediastinal tumor caused by immunoglobulin G4‐related disease and avoid the need for postoperative medication: A case report

Uchida

Matsubara

Nagasaka

et al. 2018

Asian J Endoscop Surgery

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Immunoglobulin (Ig) G4-related disease has various clinical signs and symptoms, and steroidal therapy with corticosteroids has been found to be effective for treatment. Few cases of IgG4-related disease associated with paravertebral tumor have been reported, and there have been no reports on complete resection of such a tumor. Here, we report a case of IgG4-related disease associated with a paravertebral tumor that was successfully resected without the need for postoperative medication. An 84-year-old woman was admitted to our hospital with a paravertebral tumor. She underwent thoracoscopic surgery, and pathological examination of the tumor specimen revealed that the tumor resulted from IgG4-related disease. After resection, there was no need for postoperative medication. Our case indicates the rare possibility of a paravertebral tumor associated with IgG4-related disease and the potential for complete resection as a treatment for such a tumor.

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Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

Cited by 8 publications

References 28 publications

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions

Video‐assisted thoracoscopic surgery can help enable the complete resection of a mediastinal tumor caused by immunoglobulin G4‐related disease and avoid the need for postoperative medication: A case report

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