Radiological and clinical findings of osseous peripheral primitive neuroectodermal tumors

摘要

Peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant undifferentiated type of tumor. The aim of the present study was to analyze the computed tomography (CT), magnetic resonance imaging (MRI) and clinical findings of osseous pPNET. The present study retrospectively analyzed the clinical data and CT findings from 15 patients with osseous pPNET; the MRI findings from 11 of these 15 patients were confirmed by histopathological examination. The 15 patients included 9 men and 6 women. The mean patient age was 29 years (range, 16–64 years) and 11 cases were aged <30 years. A CT scan was performed in 15 cases and the findings included a lytic lesion (13 cases), a lytic lesion with irregular sclerosis and dilation (2 cases), a soft tissue mass (15 cases), calcification (2 cases) and periosteal reaction (5 cases). A total of 9 cases of soft tissue mass were heterogeneous, with different sizes of lower-density necrotic areas. An enhanced MRI scan was performed in 11 cases. On T1-weighted images (WI), the soft tissue mass was isointense (8 cases) and marginally hyperintense (3 cases). On T2WI, aggressive soft tissue masses were heterogeneous iso- or hyperintense (11 cases). On contrast-enhanced T1WI, marked heterogeneous enhancement was present in 10 cases and intermediate heterogeneous enhancement in 1 case. The results indicated that osseous pPNET mainly affects male adolescents and young adults. The CT findings of osseous pPNET were destructive lesions with a soft tissue mass and, occasionally, with periosteal reaction. The tumor was often isodense, with patchy hypodense areas. Tumor calcification was uncommon. The MRI findings were those of an aggressive soft tissue mass exhibiting isointensity on T1WI and iso- or hyperintensity on T2WI, with marked heterogeneous enhancement. Although the imaging characteristics of pPNETs may be non-specific, CT and MRI may be useful in delineating the extent of the tumor, identifying distant metastases, predicting resectability and monitoring treatment.