BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare syndrome.The treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic patients.The conventional concept ho...BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare syndrome.The treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic patients.The conventional concept holds that the natural course of non-ossifying fibromas(NOFs)grows with the development of bones,and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the lesion.But in this case,the bone lesions were potentially biologically aggressive,which led to severe limb deformities and pain.CASE SUMMARY We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being touched.Skin examination revealed multiple café-au-lait macules on the neck,arm,axilla,and torso,including the nipples and perineum.Radiographs revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and fibula.Curettage and biopsy were performed on the distal part of the right femur.At the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica casting.At the age of 10,the girl came to our hospital again for severe pain of the right leg.Amputation from the middle level of the right femur was performed.We present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stret展开更多
目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5～69岁,平均34.56岁。74.36%的病例累及上肢...目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5～69岁,平均34.56岁。74.36%的病例累及上肢,尤以掌指部为主。临床上,66.67%病例表现为局部无痛性缓慢生长的肿块,33.33%病例伴局部压痛、肿痛症状。10.26%病例曾有局部外伤史,患者术后复发率为14.29%。以MRI为代表的影像学成像显示病变与肌腱、腱鞘或关节囊紧密相连。大体上,病变包膜纤薄或无包膜。镜下病变呈分叶状,每个小叶由稀疏散在的纤维母细胞、裂隙状血管腔隙和大量玻璃样变胶原间质所组成。部分病变形态多样,局部与促结缔组织增生性纤维母细胞瘤、纤维组织细胞瘤或纤维瘤病、结节性筋膜炎及微静脉型血管瘤病变相似。免疫组化示所有成分均表达vimentin,部分梭形及星形细胞表达α-SMA、calponin及CD163,少数病变局灶弱阳性表达β-catenin(胞质)、CD68、MSA及h-Caldesmon,裂隙状腔隙内衬扁平细胞表达CD34、CD31及FⅧRAg,提示为血管性腔隙。结论 FTS是一种相对少见的发生于肌腱、腱鞘或关节滑囊的良性纤维母/肌纤维母细胞增生性病变。典型的FTS多发生于青年人及成年人,以男性居多,好发于上肢,掌指部占多数,少数病变切除后可复发。展开更多
文摘BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare syndrome.The treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic patients.The conventional concept holds that the natural course of non-ossifying fibromas(NOFs)grows with the development of bones,and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the lesion.But in this case,the bone lesions were potentially biologically aggressive,which led to severe limb deformities and pain.CASE SUMMARY We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being touched.Skin examination revealed multiple café-au-lait macules on the neck,arm,axilla,and torso,including the nipples and perineum.Radiographs revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and fibula.Curettage and biopsy were performed on the distal part of the right femur.At the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica casting.At the age of 10,the girl came to our hospital again for severe pain of the right leg.Amputation from the middle level of the right femur was performed.We present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stret
文摘目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5～69岁,平均34.56岁。74.36%的病例累及上肢,尤以掌指部为主。临床上,66.67%病例表现为局部无痛性缓慢生长的肿块,33.33%病例伴局部压痛、肿痛症状。10.26%病例曾有局部外伤史,患者术后复发率为14.29%。以MRI为代表的影像学成像显示病变与肌腱、腱鞘或关节囊紧密相连。大体上,病变包膜纤薄或无包膜。镜下病变呈分叶状,每个小叶由稀疏散在的纤维母细胞、裂隙状血管腔隙和大量玻璃样变胶原间质所组成。部分病变形态多样,局部与促结缔组织增生性纤维母细胞瘤、纤维组织细胞瘤或纤维瘤病、结节性筋膜炎及微静脉型血管瘤病变相似。免疫组化示所有成分均表达vimentin,部分梭形及星形细胞表达α-SMA、calponin及CD163,少数病变局灶弱阳性表达β-catenin(胞质)、CD68、MSA及h-Caldesmon,裂隙状腔隙内衬扁平细胞表达CD34、CD31及FⅧRAg,提示为血管性腔隙。结论 FTS是一种相对少见的发生于肌腱、腱鞘或关节滑囊的良性纤维母/肌纤维母细胞增生性病变。典型的FTS多发生于青年人及成年人,以男性居多,好发于上肢,掌指部占多数,少数病变切除后可复发。
