Acute invasive fungal rhinosinusitis (AifRS) can spread beyond the sinonasal cavity. it is necessary to analyze the association between the specific site involved in the extrasinonasal area and the survival rate to predict patient prognosis. We investigated 50 patients who had extrasinonasal lesions on preoperative gadolinium (Gd)-enhanced magnetic resonance imaging (MRi) scan and underwent wide surgical resection of AIFRS. The specific sites with loss of contrast enhancement (LoCE) on Gd-enhanced MRI were analyzed for AIFRS-specific survival rate. The most common underlying disease was diabetes mellitus followed by hematological malignancy. the most common symptoms were headache and facial pain. Seven patients (14.0%) expired because of AIFRS progression. Poor prognosis was independently associated with Loce at the skull base on preoperative MRi (HR = 35.846, P = 0.004). In patients with AifRS extending to the extrasinonasal area, Loce at the skull base was an independent poor prognostic factor. Acute invasive fungal rhinosinusitis (AIFRS) is a rare but fatal disease, particularly in immune-compromised patients, including those with hematological malignancies, poorly controlled diabetes mellitus (DM), and those who received immunosuppressive treatment following organ transplantation or chemotherapy for solid organ malignancies 1-3. Surgical debridement, systemic antifungal therapy, and prompt correction of the underlying systemic disease are necessary for treatment of AIFRS 4,5. A recent systematic review of AIFRS reported a mortality rate of 49.7% that advanced age and intracranial involvement were independent poor prognostic factors, and that DM and surgical resection were good prognostic factors 6. Surgical resection of necrotic tissue is an important treatment modality for AIFRS; therefore, the extent of AIFRS may be a significant prognostic factor. Recently, it has been shown that information from gadolinium (Gd)-enhanced magnetic resonance imaging (MRI) may be helpful in predicting prognosis 7. Loss of contrast enhancement (LoCE) on MRI reveals tissue infarction secondary to vessel invasion by a fungus 8-10 , which cannot be infiltrated by antifungal agents and requires surgical resection. However, the surgeon may be reluctant to remove the extrasinonasal lesion if there is a lesion on the site that is likely to cause serious postoperative complications, including cerebrospinal fluid (CSF) leakage and large vessel injury. Therefore, analyzing the relationship between the specific site of involvement in the extrasinonasal area and the survival rate is important in predicting patient prognosis. To the best of our knowledge, there are no previous reports on the relationship between specific sites with LoCE on Gd-enhanced MRI and prognosis of AIFRS. In the present study, we aimed to analyze the clinical features of patients with AIFRS extending to the extrasinonasal area and to identify prognostic factors for AIFRS-specific survival rate in terms of specific sites involved. Results Headache and facial pain are...