腹部促纤维结缔组织增生性小圆细胞瘤CT表现及病理对照

Intra-abdominal desmoplastic small round cell tumors:CT findings and pathological correlations in 8 cases

目的:探讨腹部促结缔组织增生性小圆细胞肿瘤(DSRCT)的CT表现。方法:回顾性分析经病理证实的8例DSRCT患者的CT表现,8例患者均行CT增强扫描,并对其CT表现与病理学进行对照分析。结果:3例DSRCT单发,5例多发;病变主要位于膀胱后方(n=5)及肠系膜间隙(n=5);CT表现为腹、盆腔内分叶状或结节状低密度肿块;可见坏死区(n=3)及钙化灶(n=4);增强扫描均表现为不均匀强化,平扫、增强扫描动脉期及静脉期肿块的平均CT值各为38.9 HU5、6.1 HU6、2.5 HU。8例肿瘤病理特点均表现为瘤组织呈片状或巢状弥漫浸润性生长,中间由宽厚的纤维结缔组织分隔,3例可见瘤内坏死。结论:DSRCT的CT表现与病理之间存在一定的相关性,CT有助于DSRCT的诊断、分期及定位活检。

Objective：To investigate the CT findings in desmoplastic small round cell tumors（DSRCT）.Methods：8 cases with pathologically proved hepatic DSRCT underwent plain and enhanced CT scans,the imaging features were analysed retrospectively and their correlations with pathology were studied.Results：All 8 cases of DSRCT demonstrated solitary lesions（n=5） or multiple irregular masses（n=5）.The main sites of peritoneal involvement were the pelvic space（n=5） and omentum（n=5）.CT showed lobulated or nodular masses with heterogeneous hypodensity in the abdominopelvic spaces.Intratumoral necrosis（n=3） and calcifications（n=4） could be noted.On contrast enhanced CT scan,all lesions revealed heterogeneous enhancement.The mean CT attenuation values of the tumors at precontrast phase,arterial phase and portal phase were 38.9HU,56.1HU and 62.5HU respectively.Microscopic analysis of all 8 cases demonstrated clumps of small round cells with hyperchromatic nuclei and small eosinophilic cytoplasm,surrounded by a hypocellular desmoplastic,collagenous stroma.Three of them showed intratumoral necrosis.Conclusion：There are certain relations between spiral CT appearance and histopathology in DSRCT.CT Imaging is useful for diagnosis of DSRCT and its staging and also for guiding biopsies.