ObjectiveTo analyze the clinical features, diagnosis and differential diagnosis of peripheral primary neuroectodermal tumor(pPNET).

MethodsThe clinical data and diagnosis of a patient with rapid progressive pPNET in Shandong Provincial Hospital affiliated to Shandong University in January 2016 was reported and the related literatures were reviewed.The literature reviews were carried out respectively in CNKI, Wanfang and PubMed by July 2016 with "primitive neurotodermal tumour" and "PNET" being the search term from March 1994 to July 2016, including 13 articles.

ResultsA 41 year-old male patient was admitted to the hospital because of shortness of breath and occasional chest tightness, accompanied by general asthenia of about 15 d. Positron emission and transmission-CT of total trunk showed a mass in the right femoral osteoperiosteal mass, and multiple nodules in the left lung, lumps and nodules in the right lung, and right pleural thickening and effusion. Thoracoscopy was performed and pathology study confirmed the diagnosis of peripheral primary neuroectodermal tumor. The patient was given 2 courses of chemotherapy, but had rapid progressive worsening and died 1 month after PNET diagnosis. A total of 13 literatures of PNET were retrieved, all of which were case reports, and a total of 15 cases were reported.There were 9 male and 7 female patients, with a median age of 26 years.The symptoms had no specificity and most of them were solid masses in chest images, with or without pleural effusion. Eight cases were diagnosed by imaging guided percutaneous biopsy, 7 by operation and pathology, and 1 by medical thoracoscopy. Four cases underwent chemotherapy, and the survival time was less than 6 months. Twelve cases got surgical resection: 2 with surgical treatment, 7 with postoperative chemotherapy, 2 with postoperative radiotherapy and chemotherapy, 1 with postoperative chemotherapy and autologous bone marrow transplantation treatment, among which 11 patients completed follow-up. During follow-up, 3 cases died, and the survival time was 10 months, 3 years and 7 years, respectively.

ConclusionsPNET is rare.Due to the lack of specificity of clinical manifestations, clinical diagnosis depends on biopsy. Early diagnosis and surgical resection are especially important for prognosis and quality of life.