Josephson Gary D, Patel Sapna, Duckworth Laurie, Goldstein Jeffrey
Division of Pediatric Otolaryngology-Head and Neck Surgery, Nemours Children's Clinic, Jacksonville, FL 32225, USA.
Int J Pediatr Otorhinolaryngol. 2010 Dec;74(12):1452-4. doi: 10.1016/j.ijporl.2010.09.015. Epub 2010 Oct 14.
Infantile myofibroma is the most common fibrous tumor of infancy. Although the etiology is unknown, some cases are associated with a genetic inheritance of variable penetrance. With a myriad of possible locations, the presenting symptoms and findings are highly variable making its diagnosis difficult. Worldwide, there have been close to three hundred reported cases. Approximately 90% of these cases presented before age 2 with over half present at birth. The incidence is likely to be greater as many asymptomatic or hidden lesions will regress prior to discovery. The following is a report on a 7 month old male diagnosed with an infantile myofibroma within his right nasal cavity originating from the anterior skull base. This case is unusual in both its symptom presentation as well as location of origin of this tumor.
婴儿肌纤维瘤是婴儿期最常见的纤维性肿瘤。尽管病因不明,但一些病例与具有可变外显率的遗传遗传有关。由于可能的发病部位众多,其临床表现和检查结果差异很大,难以诊断。在全球范围内,已有近300例报告病例。其中约90%的病例在2岁前出现,超过一半在出生时就已存在。由于许多无症状或隐匿性病变在发现前会自行消退,其发病率可能更高。以下是一例报告,一名7个月大的男性被诊断出右鼻腔内患有起源于前颅底的婴儿肌纤维瘤。该病例在症状表现和肿瘤起源部位两方面均较为罕见。