A Case Report Of Primary Ewing’s Sarcoma/Pnet Of The Lung And Literatures Systemic Analysis

Objective:Analyze the clinical features, diagnosis and therapy of primary Ewing’s Sarcoma/Primitive neuroectodermal tumours (ES/PNET) of the lung; to improve the strategy of the diagnosis and therapy of ES/PNET of the lung.Method:Retrospectively review of a case of primary ES/PNET of the lung administrated in the first affiliated hospital of Guangxi Medical University in 2011.By retrieving the literatures using the databases such as the Chinese hownet and Pubmed, and combined with the literature systemic analysis its clinical manifestation and therapy.Result:(1)Data in this case:①General information:the Patient was a female,aged 52,famer;②Clinical manifestations:presenting symptoms included cough、right chest pain and shortness of breath;physical examination:A solitary enlarged left neck lymph node was palpable about 0.5*0.5cm,well activity,no tenderness. Auscultation over the chest showed the vocal resonance on the right lung was abated,the lower right lung percussive flatness and the breath sounds disappeared;The right upper lung breath sounds crude, audible a little wet rale.③The radiological features of the lung:CT revealed a large,irregularly marginated,and heterogeneous mass with multiple hypodense areas involving the whole right hemithorax,and few specks of calcification seen within. And also have several lesions in the rest of lung lobe.④ulmonary funcati on:Severe mixed ventilation dysfunction,moderate to severe airway obstruction.⑤Positron emission tomography(PET):a large lesions about 133*81*162mm in the right lung. And the FDG uptake was increased in the lesions. And in the rest of lung lobe there was also several lesions with high FDG uptake. The rest of the parts did not see clear metastases. ⑥The pathologic result of the lung:a small round cell tumor. Immunohistochemical:CD99(±)、NSE (+) 、CgA (-) 、Syn(±)、 CD56 (+). And all the results of immunohistochemical staining support the diagnosis of EES/PNET. ⑦Treatment:In April 2011 the patient underwent 6 cycles of chemotherapy consisting of ifosfamide,cis-platinum and etoposide During this period the CT showed lesions were absorbed. So she stopped chemotherapy in August 2011. But the lesions was increased in February, 2012,and the patient got a cycle of chemotherapy at that time with the same chemotherapy regimens. We lost to follow-up in 2013 because of tumor recurrence.(2) A systemic analysis of related literatures:Since 1998,Only 35 cases was reported all the world,and most of them are case report. ①general condition:the patients were in the age group of 8-67y and 19 of the 35 cases were males(M:F=1.2:1),and there is a peak incidence of diagnosis between the ages of 20 and 29 years,the average age of 29.9 years.②The most common clinical manifestations are chest pain, cough, fever, some patients aslo have shortness of breathy hemoptysis 、and a few of them haven’t symptoms.③The lung radiological features:the lung revealed a single large, irregularly marginated,and heterogeneous mass with uneven density.④Histologically,all of the tumour consists of small round cells. Immunohistochemical:CD99 positive rate was 94.3% and was a specific antibody,most of patient also positive express NSE、 vime,a few of patient can express syn、Ki-67、CgA、CD56、S-100.⑤The disease progress quickly,easy to relapsethe,20 of the 33 cases were dead.⑥Multimodality treatment, which includes surgical resection, and chemotherapy, and irradiation, is the treatment of first choice for these tumors, and whose survival time were longger than only chemotherapy.The first choice to no transfer of patients is early surgery combined chemotherapy and radiotherapy, however, to transfer of patients chemotherapy combined radiotherapy is given first.The chemotherapy consisted of alternating cycles every 3 weeks of cyclophosphamide, doxorobucin and vincristine, with isofosfamide and etoposide, respectively,which should be completed at last 7cycles.Conclusion: ①ulmonary Ewing’s sarcoma/primitive neuroectodermal tumor is a short course, rapidly proceeded, poor prognosis and rare disease.And often happens at young and middle-aged, the average age is 29.9 years. Presenting symptoms included cough, chest pain and fever, some patient haven’t symptoms.②Chest imaging showed a lonely, large and uneven density mass in the lung, and consisted of the pathological characteristics that histologically is a small round cells tumor, immunohistochemical is positivity with CD99 、NSE and Vime, which are the main diagnostic methods. But need except other part of the ES/PNET transferred to the lung. ③Comprehensive treatment is the first choice, to no transfer need give priority with surgery, to transfer first choice is chemotherapy.The chemotherapy consisted of alternating cycles every 3 weeks of CVA/IE,which should be completed at last 7cycles.