背景:口腔颌面部肌纤维瘤/肌纤维瘤病的影像学表现因患者而异。尽管已经报道了许多临床病例,目前对本病的临床病理特征和治疗原则缺乏共识.
目的:本研究旨在总结儿童口腔颌面部孤立性肌纤维瘤的临床病理特征。
方法:临床资料,组织学特征,收集10例接受手术切除并随后病理诊断为肌纤维瘤的儿科患者的免疫组织化学特征,并进行回顾性和横断面分析。
结果:7名患者为男性,三个是女性,年龄从3个月到6岁不等(平均:2.6岁)。患者表现为累及下颌牙龈和邻近下颌骨的孤立性病变(4例),下颌骨(2例),口腔底和下颌下区及相邻下颌骨(1例患者),牙龈(1名患者),上颌骨(1名患者),口咽(1名患者)。光学显微镜显示梭形肿瘤细胞以束或涡旋模式组织,形成血管外皮细胞瘤样血管周模式,而免疫组织化学染色显示弥漫性平滑肌肌动蛋白(SMA)阳性。所有患者均行手术切除,在12至82个月的随访期间,无一例复发.
结论:口腔颌面部孤立性肌纤维瘤主要见于婴幼儿,男性发病率较高。下颌骨病变局部切除或刮除后预后良好。准确识别临床,放射学,病理变化将降低误诊率。
BACKGROUND: The imaging manifestations of oral and maxillofacial myofibroma/
myofibromatosis can vary among patients. Although many clinical cases have been reported, a consensus on the clinicopathological features of and treatment principles for this disease is lacking.
OBJECTIVE: This study aimed to summarize the clinicopathological features of solitary myofibroma of the oral and maxillofacial regions in pediatric patients.
METHODS: The clinical data, histological features, and immunohistochemical characteristics of ten pediatric patients who underwent surgical removal and subsequent pathological diagnosis of myofibroma were collected and retrospectively and cross-sectionally analyzed.
RESULTS: Seven patients were male, and 3 were female, with ages ranging from 3 months to 6 years (mean: 2.6 years). The patients presented with solitary lesions involving the mandibular gingiva and adjacent mandible (4 patients), mandible (2 patients), oral floor and submandibular area and adjacent mandible (1 patient), gingiva (1 patient), maxilla (1 patient), and oropharynx (1 patient). Light microscopy revealed spindle-shaped tumor cells organized in bundles or vortex patterns, forming a hemangiopericytoma-like perivascular pattern, whereas immunohistochemical staining revealed diffuse smooth muscle actin (SMA) positivity. All patients underwent surgical resection, and none experienced recurrence over the 12- to 82-month follow-up.
CONCLUSIONS: Solitary myofibroma in the oral and maxillofacial regions is predominantly observed in infants and young children, with a higher incidence among males. The prognosis is favorable following localized lesion resection or curettage of jawbone lesions. Accurate recognition of the clinical, radiological, and pathological features of the disease will reduce the misdiagnosis rate.