Thoracic primitive neuroectodermal tumor / 中华胸心血管外科杂志

ABSTRACT

Objective To enhance the understanding of chest primitive neuroectodermal tumor (PNET) and improve the results of management. Methods The results of 10 cases of chest PNET, which had been surgically treated and pathologically confirmed from 1999 to 2004 were retrospectively analyzed. Results There were 6 intrathoracic tumors, 2 in the chest wall, 1 in the pericardium, and 1 in the posterior mediastinum. Two intrathoracic tumors were completely resected, 2 partially resected, tumor and the involved lung were removed in 2 cases. Two had tumor with the affected ribs removed and the chest wall reconstructed. Tumor and the invaded pericardium were removed in 1 case. The PNET in the posterior mediastinum, invading into spinal canal as a bell tumor, was excised by the thoracic surgeon cooperating with neurosurgeon. There was no hospitalization death. All the specimens demonstrated positive reaction to CD99 and negative to LCA. 4 cases received postoperatively adjuvant chemotherapy, 4 radiotherapy and chemotherapy, the other 2 cases had none adjuvant therapy. 7 patients died during 6 years of follow-up, with 9 to 27 months survival. 3 patients are alive and have over 12 months survival so far. Conclusion PNET is an aggressive and invasive malignant tumor with poor prognosis. Difficulties exist in diagnosis. Besides common microscopic examination, pathological histology and immunochemistry, even electron microscopic examination may be required in differential diagnosis. Complete removal of tumor and involved tissue plays an important role, postoperative adjuvant therapy, including radiotherapy or combination with chemotherapy, may prolong survival. Modalities that are more effective should be developed to improve the treatment results.