Tumefactive fibroinflammatory lesion presenting with head and neck fibrosclerosing lesions and orbital pseudotumors: a case report

INTRODUCTION: Tumefactive fibroinflammatory lesion is an idiopathic fibrosclerosing disorder occurring in the head and neck region. It is one of a broad spectrum of entities named inflammatory pseudotumors and, as the name suggests, it mimics a lot of diseases such as malignancies or infections. Combined with its rarity, tumefactive fibroinflammatory lesion can be a tremendous diagnostic challenge. This case report describes a case of tumefactive fibroinflammatory lesion, which was initially thought to be peri-orbital and mandibular osteomyelitis caused by Aspergillus. A lengthy work up ensued and was required to reach the final diagnosis. CASE PRESENTATION: A 64-year-old Asian man with a history of diabetes mellitus and chronic kidney disease who was on hemodialysis presented with worsening exophthalmos and relapsing trismus. He was diagnosed as “mandibular osteomyelitis” about 20 years ago. Since then he had suffered chronic relapsing exophthalmos and jaw pain with numerous medical treatments. In 2011 he was diagnosed as peri-orbital and intramandibular aspergillosis because a serum Aspergillus galactomannan assay was positive. He was treated with multiple antifungal medications to no avail. A biopsy of his orbital lesions was not revealing. After repeated biopsies, we finally concluded that the patient was suffering from tumefactive fibroinflammatory lesion. Corticosteroid therapy was initiated with prompt response. CONCLUSIONS: Tumefactive fibroinflammatory lesion is a rare inflammatory benign tumor, which mimics many inflammatory and neoplastic disorders. Conventional work up including biopsy may not lead to the diagnosis without understanding this entity. Awareness of this disorder will aid early diagnosis and treatment.