Primary primitive neuroectodermal tumor of the female reproductive system and pelvis:a clinicalpathological analysis of four cases
Objective To investigate the clinicopathological features of primary primitive neuroectodermal tumor(PNET)of the female reproductive system and pelvis.Methods A retrospective analysis was performed on the clinical and pathological data of four cases with PNET that were diagnosed at our hospital.The relevant literature was reviewed.Results The patients were aged 15-63 years with 2 cases in ovary and 1 case in uterus and pelvis,respectively.In general view,tumors were of variable sizes with a single bulky or multiplenodular appearance and ill-defined.The cut surface of those tumors had asolid,fish-like,white to pinky-grey appearance.Microscopically,tumor consisted of monotonous sheets of small,round cells with little cytoplasm and indistinct cytoplasmic borders,which were distributed in nests.The mitotic figures were common.Immunohistochemical study showed that the tumor cells were positive for CD99(4/4),Fli-1(4/4),Vimentin(4/4),CD56(2/3),NSE(3/3 locally positive)and GFAP(1/2 locally positive).The four patients all died with follow-up by 5-25 months.Conclusion Primary PNET is rare in female reproductive system.The final pathological diagnosis can be made when combined with histological morphology,immunophenotype,and fluorescence in situ hybridization(FISH)detection results.At present,surgery is the main treatment.PNET has a poor prognosis,which is aggressive and apt to early metastasis.
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