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目的探讨腹盆部外周型原始神经外胚层肿瘤(peripheral primitive neuroectrodermal tumor,pPNET)的CT表现,旨在提高对该病的认识及鉴别诊断能力。方法回顾性分析15例经病理证实的腹盆部pPNET的CT表现。影像观察指标为病灶的位置、形态、大小、密度、强化形式及伴随征象。结果 15例中,肿瘤位于盆腔7例,腹腔5例,腹膜后3例。肿瘤大小约4 cm×6 cm～13 cm×18 cm,圆形或椭圆形者8例,分叶状或不规则形7例。6例肿瘤边界清楚,9例肿瘤边界不清并累及相邻器官。平扫肿瘤呈等、低混杂密度,4例伴坏死、囊变,2例病灶可见点状、斑片状或不规则形钙化。增强后病灶均呈轻度或中度不均匀强化。结论腹盆部pPNET的CT表现多样、缺乏特异性,需与腹盆腔的胃肠道间质瘤和间叶组织肿瘤进行鉴别,确诊仍需依赖病理学诊断。
Objective To investigate the CT findings of peripheral primitive neuroectrodermal tumor (pPNET) in the abdomen pelvis to improve the understanding and differential diagnosis of the disease. Methods The CT findings of 15 cases of pathologically confirmed pelvic pPNET were retrospectively analyzed. Imaging observation of the lesion location, shape, size, density, enhanced form and accompanying signs. Results In 15 cases, the tumor was located in pelvic cavity in 7 cases, abdominal cavity in 5 cases and retroperitoneal in 3 cases. Tumor size of about 4 cm × 6 cm ~ 13 cm × 18 cm, circular or oval in 8 cases, lobulated or irregular shape in 7 cases. Six cases had clear tumor boundaries, nine had unclear border and involved adjacent organs. Tumors were equal, low, mixed density, 4 patients with necrosis, cystic change, 2 lesions showed punctate, patchy or irregular calcification. After enhanced lesions were mild or moderate non-uniform enhancement. Conclusions The CT findings of pPNET in the abdomen basin are diverse and lack of specificity. They should be differentiated from the gastrointestinal stromal tumors and mesenchymal tumors of the abdominal pelvis. Diagnosis of pathological diagnosis is still required.