Ibarburen C, Haberman J J, Zerhouni E A
Department of Radiology, Mostoles Hospital, Madrid, Spain.
Eur J Radiol. 1996 Feb;21(3):225-32. doi: 10.1016/0720-048X(95)00731-5.
The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment.
在一项回顾性研究中，对17例确诊为外周原始神经外胚层肿瘤（PNET）患者的临床、影像学和病理特征进行了评估。诊断时的年龄范围为9个月至46岁（中位数为15.8岁）。主要受累部位为腹部（n = 8）、四肢（n = 5）、胸部（n = 1）、颞骨（n = 1）、上颌骨（n = 1）和板障（n = 1）。诊断时，6例患者有远处转移；所有这些患者均死亡，平均生存期为8.8个月。影像学检查包括标准X线片、超声、计算机断层扫描（CT）、磁共振成像（MRI）和骨闪烁显像。这些肿瘤的影像学表现对于将PNET与其他类型的骨和软组织肿瘤进行鉴别并无特异性。典型表现类似于具有囊性或坏死区域的大的非钙化软组织肿块。CT上静脉内造影剂增强不均匀，在T1加权图像上呈中等信号强度，在T2加权和短TI反转恢复（STIR）序列上呈高信号强度。给予钆剂后，可见不同程度的增强。MRI和CT有助于预测可切除性、检测远处转移以及评估治疗反应。所有病例均进行了手术，目的是明确诊断或进行治疗。所有患者均接受了联合化疗和放疗，5例患者接受了自体骨髓移植。平均随访期为3年（范围1天至6年）。预后较差，中位生存期为3.4年。我们对17例外周神经外胚层肿瘤患者的经验表明，尽管其影像学特征不具有特异性，但在侵袭性软组织肿瘤尤其是年轻年龄组的鉴别诊断中应考虑到它们。CT和MRI有助于描绘肿瘤范围和可切除性以及监测治疗。
