Tumefactive fibroinflammatory lesions (TFILs) of the head and neck are rare and benign but locally aggressive lesions. The etiology and pathogenesis of these lesions are unknown. Medical management is regarded the first line of treatment. Surgical management has been rarely reported for head and neck lesions. A 51-year-old female presented with a 6-month history of left facial swelling and pain that subsequently developed into progressive inframalar hollowing and asymmetry. Biopsies confirmed tumefactive fibroinflammatory lesion of the maxilla. Initial treatment with high-dose steroids led to temporary partial involution; however, symptoms progressed. Cyclophosphamide and then rituximab were commenced, with minimal response. Imaging showed progression toward the infratemporal fossa. The patient subsequently underwent a resection and microvascular free flap reconstruction This patient had a successful surgical outcome and resolution of serum inflammatory markers with no evidence of recurrence after 18-month follow-up. A multidisciplinary approach is crucial to ensure a pragmatic patient-specific management plan is developed. Surgical resection and reconstruction can be successful in these lesions and should be considered if medical therapy has failed.
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http://dx.doi.org/10.1007/s10006-020-00914-y | DOI Listing |
Asia Pac J Ophthalmol (Phila)
October 2024
Department of Ophthalmology and Visual Sciences, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China; Hong Kong Eye Hospital, Hong Kong, China. Electronic address:
Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) is an emerging, immune-mediated fibroinflammatory orbital disease, characterized by tumefactive lesions with noticeable IgG4+ plasma cell infiltration and distinctive pathohistological features. This disease is often associated with elevated serum IgG4 concentrations. IgG4-ROD may affect any ophthalmic tissues, particularly the lacrimal gland, extraocular muscles, and trigeminal nerves.
View Article and Find Full Text PDFCureus
June 2024
Radiology Department, General Hospital of Imathia, Veria Unit, Veria, GRC.
We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI.
View Article and Find Full Text PDFMod Rheumatol Case Rep
July 2024
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-driven fibroinflammatory disease that presents as tumefactive lesions that not only commonly affects the pancreas, lacrimal and salivary glands, lung, liver and kidney but can also affect any organs. However, involvement of the urinary bladder in IgG4-RD is rarely reported. We describe a case of IgG4-RD involving the urinary bladder mimicking carcinoma and review the published literature-a 39-year-old male presented with complaints of dysuria, urgency and hesitancy.
View Article and Find Full Text PDFCurr Med Imaging
February 2024
Department of Radiology, Kangwon National University, Korea, South.
Background: Immunoglobulin G4 (Ig G4)-related disease is rare; however, it is a fibroinflammatory disease that has been studied a lot so far. Although the expression pattern varies depending on the organ affected, it usually manifests as organ hypertrophy and organ dysfunction.
Case Presentation: A 46-year-old man was referred to our otorhinolaryngology department for left submandibular swelling and tenderness that occurred 2 weeks ago.
Virchows Arch
March 2024
International Head and Neck Scientific Group, 35100, Padua, Italy.
Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality.
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