假单菌性血管内皮瘤(PMH)是一种罕见的血管肿瘤,发生在年轻的男性患者中。70%的PMH病例是多灶性的,25%涉及骨骼。PMH是一种惰性肿瘤,具有轻度的局部侵袭性和不清楚的病理。仅报道了2例自发性退化骨PMH。这里,我们报道了一例17岁的男孩,患有多灶性骨PMH,诊断为左膝慢性疼痛.PMH影响了右肩胛骨,都是胡梅里,右鹰嘴,第二掌骨,右边的第二和第四根肋骨,胸椎和腰椎,骨盆环,左右股骨颈,还有左髌骨.每个病变都有分叶,裂解模式,有时有一个周围硬化的边缘。MRI显示组织病变,T1加权序列强度低,T2加权序列强度高。T1钆脂肪饱和序列的增强是明亮的。经过讨论,一个国家专门委员会决定积极监测患者,并在进展情况下开始一般化疗.该疾病在3和6个月时稳定,并在1年时显示出消退的迹象,这在2年内得到了进一步证实。CT扫描和MRI强调了肿瘤逐渐充满松质骨和组织的消退。该病例报告强调了一种新的无痛性PMH治疗方法,该方法不会阻止进展情况下的进一步治疗。
Pseudomyogenic hemangioendothelioma (PMH) is a rare vascular tumor that occurs in young mostly male patients. Seventy percent of PMH cases are multifocal and 25% involve bones. PMH is an indolent tumor with mild local aggressiveness and an unclear pathology. Only two cases of spontaneous regressive bone PMH have been reported. Here, we report the
case of a 17-year-old boy with a multifocal bone PMH diagnosed from a chronic pain in his left knee. The PMH affected the right scapula, both humeri, the right olecranon, the second metacarpal bone, the second and fourth right ribs, the thoracic and lumbar spine, the pelvic ring, the left and right femoral neck, and the left patella. Every lesion presented with a lobulated, lytic pattern, sometimes with a peripheral sclerotic rim. MRI showed a tissue lesion with a low intensity on T1-weighted sequences and high intensity on T2-weighted sequences. Enhancement of T1 gadolinium fat-saturated sequences was bright. After discussion, a national specialized board decided to actively monitor the patient and start general chemotherapy in the
case of progression. The disease was stable at 3 and 6 months and showed signs of regression at 1 year, which was further confirmed at 2 years. CT scan and MRI highlighted a progressive filling of the tumor with cancellous bone and a regression of the tissue contingent. This
case report highlights to a new therapeutic approach for indolent PMH that does not prevent further treatment in the
case of progression.