目的:婴儿肌纤维瘤病是一种罕见的儿童时期,以软组织良性肌纤维母细胞瘤为特征,骨头,偶尔还有内脏。孤立性骨骼病变相对少见。对于适当的治疗,应将颅骨受累与更具侵袭性的肿瘤区分开来。
方法:我们回顾了孤立性婴儿颅骨肌纤维瘤,并讨论了相关的计算机断层扫描和磁共振成像发现以及鉴别诊断。还介绍了一个5个月大女孩的额骨案例研究。
结果:回顾了14例病例,包括目前的情况。在已知性别的13例病例中,八人是男性,五人是女性。平均年龄为3.03,年龄范围为0.41-9岁。14个肿瘤中有9个在额骨。病变在颅骨内翻和/或外翻累及颅骨。平均最大直径为22.3mm。在计算机断层扫描时,所有的病变都是扩张性和溶解性的,和低减毒,等减毒或偶尔过度。没有看到钙化。在磁共振成像上,大多数肿瘤在T1加权和T2加权图像上为低信号。肿瘤在弥散加权成像上显示低信号,在表观弥散系数上显示高信号,在三种情况下没有限制扩散。钆给药后,所有病变均强烈增强。治疗为全手术切除,随访期间未观察到复发。
结论:婴儿肌纤维瘤很少见,通常是带外和/或外受累的体内扩张裂解性病变。独特的成像特征包括在T2加权的磁共振图像上存在高强度信号,而在扩散加权成像上没有限制的扩散。一个缓慢增长的,公司,无痛,无触痛性肿块和支持性影像学检查结果应引起对该疾病的怀疑。
OBJECTIVE: Infantile
myofibromatosis is a rare entity of childhood characterized by benign myofibroblastic tumors in the soft tissues, the bones, and occasionally the viscera. Solitary skeletal lesions are relatively uncommon. Calvarial involvement should be distinguished from more aggressive tumors for appropriate treatment.
METHODS: We reviewed solitary infantile myofibroma of the calvarium and discussed the relevant computed tomography and magnetic resonance imaging findings along with differential diagnosis. A case study of the frontal bone in a 5-month-old girl was also presented.
RESULTS: Fourteen cases were reviewed, including the current case. Of the 13 cases with known sex, eight were male and five female. The mean age was 3.03 with an age range of 0.41-9 years. Nine of the 14 tumors were in the frontal bone. The lesions were intradiploic with tabula interna and/or externa of the calvaria involvement. The mean largest diameter was 22.3 mm. Upon computed tomography, all the lesions were expansile and lytic, and hypoattenuated, isoattenuated or occasionally hyperatenuated. Calcification was not seen. On magnetic resonance imaging, most neoplasms were hypointense on T1-weighted and T2-weighted images. Neoplasms showed hypointense signal on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, without restricted diffusion in three cases. All lesions were intensely enhanced after gadolinium administration. Treatment was total surgical resection and recurrence was not observed during follow-up.
CONCLUSIONS: Infantile myofibromas are rare, typically intradiploic expansile lytic lesions with tabula interna and/or externa involvement. Distinctive imaging features include the presence of hipointense signals on T2-weighted magnetic resonance images without restricted diffusion on diffusion-weighted imaging. A slow-growing, firm, painless, and nontender mass with supportive imaging findings should raise suspicion of the disease.