| Objective：Primitive neuroectodermal tumor is a rare malignant tumor, and it is rarer infemale genital tract. However, with high malignant degree and poor prognosis, PNETis easily to experience recurrence and metastasis. The aim of the study is toinvestigate the clinical characteristics of PNET, including the incidence, clinicalmanifestation, pathological characteristics, immunohistochemical staining, treatmentmethods and the factors influencing the survival in both female genital tract and othersystems.Methods：1. Cases with complete record data and pathological results were collected fromPeking Union Medical College Hospital from January1999to August2014, and allwere confirmed by HE staining and immunohistochemical staining.2. The data of gender, age, preoperative examination, tumor markers, date ofsurgery, surgical procedures, tumor size, metastasis, pathological diagnosis,immunohistochemical, radiotherapy or chemotherapy regimens and dose, recurrencetime, follow-up time and prognosis were extracted from the cases.3. The condition and survival of the patients were achieved by reexamination,outpatient care and telephone follow-up.4.SPSS was used for the statistical analysis;Kaplan-Meier method was employedto calculate the survival rates of patients，and the facts of prognosis were analyzed;Log-rank tests were used for univariate analysis.Results：1.99PNET patients,52male patients,47female patients, the ratio is1.11:1.The average age was (29.74±15.81) y, ranging5-77y.26patients were under18y and73patients were adults.2. PNET lacked typical clinical manifestations and clinical features wereassociated with the sites of the tumors.The most common symptom was pain(41.1%), and the second was the found of a mass(28.3%).Distant metastasis occurred in13%patients at the time of diagnosis and were mainly lungs and bones.3. PNET differed in tumor size. The maximum diameter ranged from1.5cm to25cm, with an average diameter of10.4cm.Microscopic examination revealed apattern of small round blue cells with Homer-Wright rosettes. Onimmunohistochemical staining, CD99was positive on the cell membrane and NSE,Syn, CgA were also positive to some different degree. However, CK, LCA andDesmin were negative.4. The incidence of PNET in female genital tract was14.14%of all the cases andit could originate from anypart of the female genital tract. Total resection of the tumortogether with chemotherapy was the main treatment.5. Theresults of Log-rank test for univariate analysis of age, gender, size of thetumor, whether there was metastasis and chemotherapy regimen showed that theP>0.05, the results of stage and total resection showed that the P<0.05.Conclusion：1. PNET was common for children and adolescents, with the most patients intheir second and third decades, but all ages were available. Male patients were morethan female patients slightly.2.PNET lacked specific clinical manifestations and the main symptoms werepain and mass. cPNET showed high intracranial pressure and other symptoms ofnervous system, while pPNET had different symptoms in different systems.3. Metastasis was an important characteristic of PNET. Part of the patients hadmetastasis to the lungs, bones or livers at the time of diagnosis.4. Diagnosis of PNET depended on pathological and immunohistochemicalexamination. Microscopic examination revealed small blue round cells andimmunohistochemical staining revealed the expression of CD99on cell membranes.Other neural differentiation markers, such as NSE, Syn and CgA were also expressedto different degree.5. Treatment for PNET included surgery, chemotherapy and radiotherapy.Surgery combined with concurrent chemotherapy and radiotherapy was the main treatment for PNET. Chemotherapy regimen was mainly VACA±IE.6.Stage and total resection were the factors that affected the prognosis.7. Female genital tract PNET was rare and could originate from any part offemale genital tract. It lacked specific manifestation and was inclined to metastasize.Diagnosis and treatment were the same to the PNET of other part. |
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