xRead - Nasal Obstruction (September 2024) Full Articles

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ICAR SINONASAL TUMORS

disease. 1044 Early disease tends to be a perivascular inflammatory lesion containing eosinophils, plasma cells, and lymphocytes. Foci of fibrosis develop as the lesion matures until there is dense fibrous thickening with perivascular “onion skin” fibrosis and decreased inflam matory cells in the late stage, although eosinophils remain prominent. 1038,1042,1045 Concentric fibrosis is seen in all cases and is more intense in longstanding disease. 1046 There is no true granulomatous reaction, cytologic atypia, necrosis, or mitotic activity observed. 1042 IgG4 assessment is also important for further characterization of the lesion and can impact management. The treatment approach for EAF may involve surgery, medical therapy, or both. A systematic review reported 90% of patients received complete or partial surgical resec tion and one third received additional medical treatment with corticosteroids or immunosuppressants. 1036 Because of the rarity of the disease, testing and comparing treat ments have been limited. The only treatment approach that has resulted in resolution of disease is total resection. The endoscopic surgical approach is the only variable that has correlated with likelihood of total resection. 1036 There is no clear benefit of adjuvant medical therapy along with complete resection in preventing recurrence. 1036,1037 Inall cases treated with partial resection with or without med ical management or medical management alone, patients had persistent disease that needed closer monitoring for progression. 1036 There have been small case reports and case series on alternative treatments, including rituximab that is known to produce a good response in IgG4RD and showed reduc tion in tumor size of 50% with symptom resolution in a case of EAF that had recurred multiple times and failed multiple debulking procedures and steroid therapy. 1041 Additionally, a recent case study showed benefit with methotrexate in EAF of the lip and palate in a single case. 1047 So while resolution of disease has only been seen with complete resection, alternative therapies may have a role when total resection is not possible or when there have been multiple recurrences. Multiple resections may be required as complete excision can be difficult as demonstrated by high recurrence rates of up to 70% fol lowing total resection. 1037,1038 Recurrence generally occurs in the original anatomic location; however, there have been no statistically significant risk factors found to predict recurrence. 1037 All EAF cases to date have been nonfa tal with no reports of malignant progression. Long-term follow-up is necessary due to the slow progression and high recurrence rate. 1039 Other benign sinonasal lesions that have been pre viously covered in ICSB include fibroosseous lesions (Section V.B), cholesterol granuloma (Section V.C), and schwannomas (Section V.D). 5

ceded by multiple recurrences, and long-term surveillance with nasal endoscopy and imaging is recommended.

G Eosinophilic angiocentric fibrosis Eosinophilic angiocentric fibrosis (EAF) is a rare indo lent fibroinflammatory lesion that typically involves the sinonasal tract, upper airway, and occasionally the orbit. The etiology of EAF is unknown, although it has been associated with allergies, atopy, chronic rhinosinusitis (CRS), prior trauma, and history of nasal surgery. 1036 Presenting symptoms are nonspecific and related to the anatomic location of the lesion, with the septum being the most common site. Nasal obstruction is found in over 75% of cases, with other presenting symptoms including nasal swelling/deformity, epistaxis, epiphora, pain, rhin orrhea, and headache. 1037 EAF was originally described as a mucosal form of granuloma faciale (GF), and while they have since been described as separate diseases, about 25% of patients with EAF have concurrent GF. 1038 There is no sex predilection, and average age of diagnosis is 48 years. 1039 Patients report symptoms for an average of 3 years prior to diagnosis. 1036 There are fewer than 70 cases of EAF described in the literature, so much remains unknown. EAF lesions possess several characteristics of IgG4 related disorders (IgG4RD), including tumefactive lesions, elevated IgG4 plasma cells, and an increased IgG4:IgG ratio. 1040 Oligoclonal expansion of T cells plays a role in IgG4RD pro-fibrosis and has also been observed in EAF. 1041 Eosinophils are not as common in IgG4RD and are more commonly seen in immune hypersensitivity production of inflammatory mediators and downstream fibroblast stimulation. 1042 However, the association with IgG4 is not absolute, with many cases not meeting the tra ditional IgG4RD criteria, although some speculate that this is because IgG4 dissipates in more progressed or chronic disease. 1036 CT and MRI characteristics in EAF are nonspecific. EAF may appear as a well-circumscribed submucosal thicken ing of the septum or lateral walls or sinus opacification. 1038 Noncontrast CT typically shows a homogenous isodensity with rare to no calcifications and includes bone thinning, remodeling, or sclerosis in about 25% of patients. 1037,1043 EAF is often misdiagnosed as inflammation or malignancy on PET because of the hypermetabolic FDG avidity. 1041 For MRI, T1 images are isointense with moderate contrast enhancement, while T2 images are hypointense, attributed to later stage fibrosis. 1043 EAF lesions grossly appear as a fleshy, tan to pink sub mucosal mass. 1042 Roberts and McCann initially described the histology and staging criteria for early and late