Chronic Invasive Fungal Rhinosinusitis with Atypical Clinical Presentation in an Immunocompromised Patient

Abstract

Invasive fungal rhinosinusitis (FRS) is a rare but intractable infectious disease of the sinonasal region with destructive direct infiltration into surrounding tissues, such as the bone, orbit and brain, and potential dissemination to systemic organs. Symptomatic assessments and imaging are frequently not sufficiently diagnostic, and histopathological examination is essential for definite diagnosis of FRS. We herein report a case of chronic invasive FRS (CIFRS) in a 58-year-old Japanese male with end-stage diabetic nephropathy that required maintenance dialysis after graft rejection of living kidney transplantation. His initial main clinical presentation was sinus gangrene, which gradually progressed from the paranasal sinus to the nasal septum and oral palate, but not towards the intracranial or orbital region, for two months. The patient was first strongly suspected to have extranodal natural killer/T cell lymphoma (ENKTL), nasal type, a subtype of malignant lymphoma, based on the macroscopic appearance of the gangrene, expansion pattern and high serum soluble interleukin-2 level; however, repeated biopsies and eventual resection led to diagnosis of CIFRS due to Aspergillus niger and Mucor. The disease was improved by surgical resection in combination with antifungal pharmacologic treatment with liposomal amphotericin B and voriconazole. CIFRS typically occurs in immunocompetent patients and shows intracranial progression, but this case shows that atypical CIFRS with an uncommon expansion pattern can occur in an immunodeficient patient.

Keywords:

• chronic invasive fungal rhinosinusitis
• diabetes mellitus
• maintenance dialysis
• extranodal natural killer/T cell lymphoma
• nasal type

Ethics and Consent Statement

Informed consent has been provided by the patient for the publication of the case report and accompanying images. The institutional approval was not required for the publication of the case details.

Disclosure

Taku Tsukamoto has received research funding from Nippon Shinyaku. Tsutomu Kobayashi has received honoraria from Chugai Pharmaceutical, Ono Pharmaceutical, Eisai, and Nippon Shinyaku. Junya Kuroda has received research funding from Celgene, Kyowa Kirin, Chugai Pharmaceutical, Ono Pharmaceutical, Sanofi, Eisai, Bristol-Myers Squibb, Sysmex, Astellas Pharma, Pfizer, Dainippon Sumitomo Pharma, Nippon Shinyaku, Takeda, Shionogi, Asahi Kasei, Daiichi Sankyo, MSD, Taiho Pharmaceutical, Fujimoto Pharmaceutical and Otsuka Pharmaceutical; honoraria from Janssen Pharmaceutical K.K, Celgene Corporation, Kyowa Kirin, Chugai Pharmaceutical, Ono Pharmaceutical, Takeda, Sanofi, Eisai, Bristol-Myers Squibb, Astellas Pharma, Pfizer, Nippon Shinyaku, Dainippon Sumitomo Pharma, Daiichi Sankyo, Fujimoto Pharmaceutical, Abbvie and Otsuka Pharmaceutical; and is a consultant for Janssen Pharmaceutical K.K, Celgene, Bristol-Myers Squibb, Sanofi and Abbvie. The authors report no other potential conflicts of interest for this work.