Khan Muhammad Tahir, Arooj Sadaf, Mukhtar Muhammad Umer, Raman Rewati
Department of Radiology, Punjab Institute of Neurosciences, Lahore, Pakistan.
King Edward Medical University, Lahore, Pakistan.
Radiol Case Rep. 2022 Aug 1;17(10):3674-3677. doi: 10.1016/j.radcr.2022.07.008. eCollection 2022 Oct.
Maffucci syndrome is a non-hereditary congenital condition that affects the skin and skeleton. Enchondromas (benign cartilage enlargements), bone abnormalities, and venous anomalies (hemangiomas) are all symptoms. Enchondromas occur as a result of mesodermal dysplasia and have the potential to become cancerous. They are most commonly found on the phalanges and long bones. Venous abnormalities commonly manifest themselves as soft lumps or tumors on the distal extremities. A 19-year-old boy presented with swellings on his fingers and left foot since the age of 5, along with a few bluish soft tissue swellings on his left heel. Multiple expansile lytic lesions and soft tissue swellings with phleboliths were seen on X-ray. Histology confirmed the diagnosis of hemangiomas and enchondromas. Soft tissue swellings were found to have hyper echoic areas, as well as modest marginal blood flow on Doppler, which could indicate hemangiomas. Maffucci syndrome was identified, and treatment with a multidisciplinary approach was initiated. Maffucci syndrome is a rare genetic illness reported in the literature less than 200 times. The enchondromas and hemangiomas have a strong link to malignant changes, with chondrosarcomas accounting for 30% of the associated malignancies. On X-ray, enchondromas are easily identified as osteolytic lesions with cortex thinning and endosteal scalloping while color Doppler ultrasound detects the presence of hemangiomas. Phleboliths are easily identified as small calcifications on X-rays. Radiographic examinations should be considered in patients presenting with bone or soft tissue swellings for an early diagnosis of Maffucci syndrome.
马富西综合征是一种影响皮肤和骨骼的非遗传性先天性疾病。内生软骨瘤(良性软骨增大)、骨骼异常和静脉畸形(血管瘤)都是其症状。内生软骨瘤是中胚层发育异常的结果,有恶变的可能。它们最常见于指骨和长骨。静脉畸形通常表现为远端肢体的软性肿块或肿瘤。一名19岁男孩自5岁起手指和左脚出现肿胀,左脚后跟还有一些蓝色软组织肿胀。X线检查可见多个膨胀性溶骨性病变和伴有静脉石的软组织肿胀。组织学检查确诊为血管瘤和内生软骨瘤。发现软组织肿胀有高回声区,多普勒检查显示边缘有少量血流,这可能提示血管瘤。确诊为马富西综合征,并开始采用多学科方法进行治疗。马富西综合征是一种罕见的遗传疾病,文献报道少于200次。内生软骨瘤和血管瘤与恶性病变有很强的关联,软骨肉瘤占相关恶性肿瘤的30%。在X线上,内生软骨瘤很容易被识别为皮质变薄和骨内膜扇贝样改变的溶骨性病变,而彩色多普勒超声可检测到血管瘤的存在。静脉石在X线上很容易被识别为小钙化。对于出现骨骼或软组织肿胀的患者,应考虑进行影像学检查以早期诊断马富西综合征。