xRead - Nasal Obstruction (September 2024) Full Articles

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ICAR SINONASAL TUMORS

was included. 969,970 Though not strictly part of the diag nostic criteria, optic nerve and/or CN V involvement is commonly associated with THS, and the involvement of CN V and the optic nerve together has been associated in a retrospective case series with longer disease duration. 971 Given the rarity of THS, the demographic distribution of THS is uncertain; however, data suggest that it may be equally distributed between males and females. 972 In THS, symptoms may improve spontaneously; how ever, they tend to remit and recur without adequate treatment. High-dose corticosteroids are the mainstay of treatment. 973 If symptoms persist despite > 72 h of high dose systemic corticosteroids, surgical exploration of the affected tissue for sampling or consideration for alternative diagnoses are warranted. 974 Aggregate grade of evidence : C (Level 2: one study; Level 4: two studies; Level 5: one study) Rosai–Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, classically presents with neck lymphadenopathy, fever, and weight loss. 979 This rare condition, linked to gene mutations in NRAS , KRAS , MAP2K1 , and ARAF , is more common in men and African Americans. 980 Usually diagnosed by the second decade, RDD can occur in isolation or with autoimmune or malig nant diseases. Isolated extranodal involvement arises in 43% of patients, with the head and neck region most affected, predominantly in the nasal cavity and paranasal sinuses (11%). 980–982 Sinonasal disease presents as polyps, obstruction, rhinorrhea, facial asymmetry, headache, epis taxis, hyposmia, and proptosis. 982,983 Imaging includes contrasted CT of the paranasal sinuses, MRI of the orbit/brain, or FDG-PET/CT. RDD lesions enhance on T1-weighted MRI and are FDG avid. 980 On IHC, RDD histocytes are S100 and OCT2 positive with variable CD14, CD68, and CD163 positivity. 980 Due to the self-limiting nature of the disease and the fact that spontaneous remission occurs in 50% of patients, manage ment is reserved for symptomatic patients. 984 Endoscopic resection of sinonasal disease can resolve symptoms. 985 For unresectable, recurrent, or multifocal disease, cor ticosteroids, chemotherapy, and immunomodulators are options; however, results are mixed. 982 RT has been suc cessful but is generally reserved for disease involving vital organs, such as the central nervous system, eyes, heart, and liver, where the risks of surgery outweigh its benefits. 981 XX OTHER RARE BENIGN NEOPLASMS AND LESIONS A Rosai–Dorfman disease

enlargement had more severe symptomology and higher propensity for recurrence. 955 As recognition of IgG4-ROD as a distinct disease entity has become more widespread, a large body of literature has focused on re-examining previously diagnosed cases of OP. Recently, Min et al. reviewed 16 cases previously diag nosed as OP, and after staining for IgG4 + plasma cells and identifying storiform fibrosis, a diagnosis of IgG4-related disease was made. 956 A review of 55 previously diagnosed cases of OP demonstrated that over 40% were histologi cally re-classified as IgG4-ROD. In another study, which evaluated cases of previously diagnosed OP, the radiologic findings of bilateral orbital involvement and infraorbital nerve involvement are associated with IgG4-ROD when tissue samples are assessed by IgG4-ROD criteria. 957,958 Corticosteroids are the first-line treatment of IgG4 ROD. 959 Data suggest that involvement of the trigeminal nerve and elevation of serum rheumatoid factor correlate with corticosteroid treatment failure, however. 960,961 In some cases, successful treatment with corticosteroids may obviate the need to obtain histopathology. 962,963 Intravenous corticosteroids have been shown to improve short-term disease, particularly when the optic nerve is involved. 964 Intraorbital corticosteroid injection alone may be safe and efficacious for anterior chamber involvement. 965 A current randomized trial comparing oral corticosteroid alone versus intraorbital steroid injec tion alone is ongoing. 966 Finally, a small body of evidence reveals that rituximab may lead to clinical and radiologic improvement in steroid-responsive and steroid-resistant IgG4-ROD. 959,967 Nevertheless, biopsy is recommended in cases that display signs of malignancy or when patients fail to respond to empiric corticosteroid therapy. Aggregate grade of evidence : C (Level 2: three stud ies; Level 3: four studies; Level 4: 13 studies; Level 5: eight studies) 2 Tolosa–Hunt syndrome THS, which was first described in 1954 Tolosa and then fur ther characterized in 1961 by Hunt, was classified in 1988 by the International Headache Society in the First Inter national Classification of Headache Disorders (ICHD-I) (Table XIX.B.2). 968 The initial criteria revolved around painful unilateral orbital pain associated with paresis of cranial nerves III, IV, or VI (inclusive of pain preceding paresis by up to 2 weeks), resolution of pain within 72 h of corticosteroid treatment, and exclusion of other causes by neuroimaging. 968 These criteria were further refined such that by the third rendition (2018) of the ICHD, gran ulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit demonstrated by MRI or biopsy