This report describes a case involving a 78-year-old Caucasian male, whose medical history was significant for sinonasal (angiocentric) T/NK lymphoma, who was treated by surgery and radiation in 1988. After the treatment, the patient was apparently free of residual/recurrent disease for a period of 12 years. There was periodical clinical follow up including repeated biopsies. After this period, the patient suffered from sudden deterioration of the health status with multiorgan involvement by the disease and he died. The diagnosis was confirmed by an autopsy. To our knowledge, this is the fifth reported case with extended survival (more than 12 years) free of recurrent/residual disease after the initial treatment, in which the patients ultimately died because of the disease. These findings suggest the importance of prolonged clinical follow-up in patients with this diagnosis.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Uncommon Fibroinflammatory Sinonasal Tract Lesions: Granulomatosis with Polyangiitis, Eosinophilic Angiocentric Fibrosis, and Rosai-Dorfman Disease.
Surg Pathol Clin
December 2024
Head and Neck Pathology Consultations, 22543 Ventura Boulevard, Suite 220 PMB1034, Woodland Hills, CA 91364, USA. Electronic address:
Fibroinflammatory lesions of the sinonasal tract include inflammatory polyps (chronic rhinosinusitis), various infectious, sarcoidosis, and NK/T-cell lymphoma as examples of the most commonly encountered lesions. However, the differential diagnosis includes several less frequently encountered entities, such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), eosinophilic angiocentric fibrosis considered part of IgG-related disease, and Rosai-Dorfman disease. This review focuses on these latter entities providing an update on clinical, laboratory, imaging, histology, and ancillary testing employed to reach an actionable diagnosis.
View Article and Find Full Text PDFUnveiling primary intracranial eosinophilic angiocentric fibrosis: A rare case report and diagnostic dilemmas.
Neuropathology
December 2024
Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Article Synopsis
- Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition that typically affects the sinonasal and upper respiratory tract, with very few cases occurring in the brain.
- The report details a case of a 55-year-old man who was initially misdiagnosed with meningioma due to similar clinical and imaging features but was confirmed to have EAF after surgical examination.
- This case highlights the importance of considering EAF in the diagnosis of brain lesions that resemble meningiomas, emphasizing the need for thorough histopathological evaluation for accurate diagnosis and treatment.
Advancements in diagnosing IgG4-related disease of the head and neck: Navigating diagnostic pitfalls.
Semin Diagn Pathol
March 2024
Harvard Medical School, Boston, MA, United States. Electronic address:
IgG4-related disease (IgG4-RD) is an immune-mediated condition affecting nearly any organ. This review focuses on the nuances of diagnosing IgG4-RD affecting the head and neck. Salivary gland involvement, especially of the submandibular glands, often permits a definitive diagnosis on biopsy.
View Article and Find Full Text PDFEosinophilic Angiocentric Fibrosis of the Orbit: A Clinicopathologic Review of 6 Novel Cases With Review of the Literature.
Am J Ophthalmol
December 2023
From the Ophthalmic Plastic Surgery, Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School (L.Y.L., V.D.L., N.G.L.). Electronic address:
Purpose: To describe 6 cases and review the current state of knowledge of eosinophilic angiocentric fibrosis (EAF) involving the orbit.
Design: Retrospective clinicopathologic case series and review of the current literature METHODS: Clinical records and histopathologic data of orbit-involving EAF were gathered between 2004 and 2022 from a single academic institution. The patients' presenting clinical symptoms and signs, laboratory data, radiographic studies, and management documentation were collected.
Eosinophilic angiocentric fibrosis of the sinonasal tract: a case report and review of the literature.
J Int Med Res
September 2022
Clinical Research Development Center, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran.
Eosinophilic angiocentric fibrosis (EAF) is a rare chronic benign disorder of unknown etiology and is characterized by submucosal thickening and fibrosis in the upper respiratory tract. In this report, we describe a case of EAF in the nasal cavity of a woman who underwent elective surgery for division of adhesions and has had no recurrence during 2 years of postoperative follow-up. A review of the literature on the clinical manifestations of EAF, sites of lesions, management, and outcomes identified 48 articles that included 72 cases.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!