Tumefactive fibroinflammatory lesions (TFLs) are rare idiopathic benign fibrosclerosing lesions that clinically simulate a malignancy. TFLs are seen more frequently in males between 10 and 74 years of age. The usual site of involvement is the head and neck region, but rarely the extremities may be involved. Coexisting fibrosclerotic processes have been reported including retroperitoneal fibrosis, sclerosing cholangitis, sclerosing mediastinal fibrosis, and orbital pseudotumors. The etiology of this poorly understood entity remains unknown. Possible suggestions include exaggerated responses or autoimmune reactions to any chronic infection. The clinical and radiological appearance of TFLs is that of malignancy, but histopathology reveals them to be a benign process broadly classified under non-neoplastic, fibroinflammatory proliferations. The treatment strategies for these lesions are not well defined and variable and include steroids, surgery, and radiotherapy either alone or in combination. TFLs, albeit not fatal, have a high recurrence rate; patients should, therefore, be kept on long-term follow-up. We describe a young female patient presenting with a rapidly developing cheek swelling, which was diagnosed histopathologically as a TFLs.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5366370 | PMC |
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.
Curr 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.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!