摘要
目的探讨法瓦病(FAVA)临床特征、组织病理特征、诊断及预后。方法观察山东省立第三医院2020年至2022年收集的6例法瓦病临床资料、组织形态特征及免疫组化表型、随访预后情况,并结合国内外文献报道进一步分析。结果6例法瓦病中,5例为女性,1例为男性。发病年龄分别为4岁、5岁、10岁、12岁、16岁、50岁。发病部位:其中2例为右下肢,2例为左下肢,1例为左前臂,1例为右股后肌间区。临床表现均为皮下缓慢生长肿物,质软,呈海绵状,分界不清,关节活动受限,伴有疼痛。磁共振显示患处可见多发不规则、条状、团块状异常信号影,T1WI呈等低信号影,T2WI及压脂序列呈不均匀高信号。镜下所见病变均位于皮下深部,肌组织内查见多量不规则的扩张血管、脂肪细胞、纤维细胞及淋巴细胞、浆细胞聚集。免疫组化染色显示6例ERG、CD31血管阳性表达,SMA骨骼肌组织阳性表达,S100脂肪组织阳性表达。随访至今,6例完全切除后,有1例半年后同一部位复发再行手术完整切除后至今未复发,其余均无复发。结论FAVA为一种少见的良性肿瘤,临床表现无明显特异性,FAVA的磁共振以及病理形态具有一定特点,提高对其组织学病理形态学特征和磁共振认识,有助于减少漏诊和误诊。
Objective To investigate the clinical features,histopathological features,diagnosis and prognosis of fibroadipose vascular anomaly(FAVA).Methods The clinical data,histological characteristics,immunohistochemical phenotype and follow-up prognosis of 6 cases of FAVA collected from Shandong Provincial Third Hospital from 2020 to 2022 were reviewed and further analyzed in combination with domestic and foreign literature.Results Among the 6 cases of FAVA,5 cases were female and 1 case was male.The age of onset were 4,5,10,12,16,and 50 years old,respectively.Site of onset:2 cases were in the right lower limb,2 cases were in the left lower limb,1 case was in the left forearm,and 1 case was in the right posterior femoral intermuscular region.The clinical manifestations were slowly growing subcutaneous masses,soft and spongy,with unclear boundaries,limited joint movement and pain.The magnetic resonance imaging showed multiple irregular,stripy and clumpy abnormal signal shadows in the affected area.T1WI showed equal low signal shadows,and T2WI and lipid-pressure sequences showed uneven high signal.Under the microscope,lesions were all located in the deep subcutaneous area,with irregular dilated blood vessels,adipocytes,fibrocytes,lymphocytes,and plasma cells aggregates observed within the muscle tissue.Immunohistochemical staining showed positive expression of ERG and CD31 in blood vessels,positive expression of SMA in skeletal muscle tissues,and positive expression of S100 in adipose tissues in the 6 cases.Up to now,among the 6 cases that have undergone complete resection surgery,1 case recurred at the same site half a year later and underwent another surgery for complete resection without recurrence so far,while the rest have not recurred.Conclusion FAVA is a rare benign tumor with no obvious clinical specificity.The magnetic resonance and pathological morphology of FAVA have certain characteristics.To improve the understanding of its histological and pathologic characteristics and magnetic resonance is helpful to reduce missed diagnosis and misdiagnosis.
作者
吕文斐
张晶
杨香山
于静
黄萌
甄军晖
LV Wenfei;ZHANG Jing;YANG Xiangshan;YU Jing;HUANG Meng;ZHEN Junhui(Department of Pathology,Shandong Provincial Third Hospital,Jinan 250031,China;School of Medicine,Shandong University,Jinan 250031,China)
出处
《诊断病理学杂志》
2025年第7期817-821,共5页
Chinese Journal of Diagnostic Pathology
基金
国家自然科学基金项目(项目编号:81972436)。
关键词
血管畸形
法瓦病
诊断
鉴别诊断
治疗
预后
Vascular malformation
FAVA disease
Diagnosis
Differential diagnosis
Treatment
Prognosis
