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目的探讨儿童外周型原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的CT、MRI表现,提高对该病的诊断水平。方法回顾性分析14例经手术病理证实的儿童pPNET的临床、CT、MRI及病理组织学表现,并复习相关文献。结果 14例患儿中,肿瘤分别位于颈部(3例),胸部(4例),脊柱旁(3例),盆腔(2例)及四肢(2例);3例肿瘤均呈不均质囊实性密度或信号,11例为实性密度或信号。CT平扫密度低于肌肉组织,未见明显钙化,周围骨性组织多可见硬化或虫蚀样破坏,部分可见骨膜反应。MR T1WI肿块呈等低信号,T2WI、短恢复时间反转恢复法(STIR)呈不均质混杂高信号,增强扫描明显不均匀强化,中心囊变、坏死区未见明显强化。结论儿童pPNET缺乏典型的特异性影像学表现,但具有一定的影像学特点。CT和MRI有助于鉴别诊断、制定手术计划。
Objective To investigate the CT and MRI findings of peripheral primitive neuroectodermal tumor (pPNET) in children and to improve the diagnosis of the disease. Methods The clinical, CT, MRI and histopathological features of 14 cases of pPNET confirmed by surgery and pathology were retrospectively reviewed. Relevant literatures were reviewed. Results Of the 14 cases, tumors were located in the neck (3 cases), chest (4 cases), paravertebral (3 cases), pelvic (2 cases) and extremities (2 cases) Solid density or signal density, 11 cases of solid density or signal. CT scan density is lower than muscle tissue, no obvious calcification, the surrounding bony tissue more visible erosion or erosion damage, and some visible periosteal reaction. MR T1WI mass showed low signal, T2WI, short recovery time reversal recovery method (STIR) showed heterogeneous mixed high signal, enhancement scan significantly uneven enhancement, central cystic degeneration, no significant enhancement. Conclusions Children pPNET lacks typical typical imaging findings, but has certain imaging features. CT and MRI help to differentiate the diagnosis, the development of surgical plans.