Kusaka Soichi, Nishimura Sho, Kawakami Fumi, Ohbayashi Chiho, Shibuya Yasuyuki, Iwata Kentaro
Division of Infectious Diseases, Kobe University Hospital, Kusunokicho 7-5-2, Chuoku, Kobe, Hyogo 650-0017, Japan.
J Med Case Rep. 2013 Nov 14;7:260. doi: 10.1186/1752-1947-7-260.
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.
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.
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.
肿炎性纤维病变是一种发生于头颈部的特发性纤维硬化性疾病。它是一系列被称为炎性假瘤的疾病之一，顾名思义，它可模仿许多疾病，如恶性肿瘤或感染。加之其罕见性，肿炎性纤维病变可能是一个巨大的诊断挑战。本病例报告描述了一例肿炎性纤维病变，最初被认为是由曲霉菌引起的眶周和下颌骨骨髓炎。经过漫长的检查过程才最终确诊。
一名64岁的亚洲男性，有糖尿病和慢性肾病病史，正在接受血液透析，出现眼球突出加重和复发性牙关紧闭。他大约20年前被诊断为“下颌骨骨髓炎”。从那时起，他经历了慢性复发性眼球突出和颌部疼痛，接受了多次治疗。2011年，他被诊断为眶周和下颌骨曲霉菌病，因为血清曲霉菌半乳甘露聚糖检测呈阳性。他接受了多种抗真菌药物治疗，但无效。对其眼眶病变进行活检未发现异常。经过多次活检，我们最终得出结论，该患者患有肿炎性纤维病变。开始使用皮质类固醇治疗后迅速见效。
肿炎性纤维病变是一种罕见的炎性良性肿瘤，可模仿许多炎性和肿瘤性疾病。如果不了解这种疾病，包括活检在内的常规检查可能无法得出诊断。认识这种疾病将有助于早期诊断和治疗。
