title: Central type primitive neuroectodermal tumor/neuroblastoma of the uterus: a case report.
abstract: We encountered a 63-year-old woman who had a uterine tumor with peritoneal dissemination and para-aortic lymph node metastasis. Microscopic specimens of the tumor showed a small blue round-cell tumor. Immunohistochemistry showed cells to be negative for cytokeratin AE1/3, desmin, myogenin, CD10, CD34, and CD99, focal positive for vimentin, and positive for muscle-specific actin (HHF-35), neurofilament, synaptophysin and CD56. Fluorescence in situ hybridization revealed no split signal showing Ewing sarcoma breakpoint region 1 gene translocation. Deletion of 1p36 was identified in 30% of the tumor cells. These findings are thought to be equivalent to central type primitive neuroectodermal tumors/neuroblastoma. Cytoreductive debulking surgery followed by chemotherapy, including cyclophosphamide, vincristine and adriamycin, resulted in complete remission. She has no evidence of disease at 24 months after surgery.
authors.affiliation: Department of Obstetrics and Gynaecology, Hokkaido University School of Medicine, Sapporo, Japan.
authors.forename: Chisa
authors.initials: C
authors.lastname: Shimada
authors.forename: Yukiharu
authors.initials: Y
authors.lastname: Todo
authors.forename: Kazuhira
authors.initials: K
authors.lastname: Okamoto
authors.forename: Daisuke
authors.initials: D
authors.lastname: Akashi
authors.forename: Katsushige
authors.initials: K
authors.lastname: Yamashiro
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hasegawa
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004317:Doxorubicin / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007044:Hysterectomy; D008207:Lymphatic Metastasis; D008875:Middle Aged; D009447:Neuroblastoma / Q000175:diagnosis* / Q000473:pathology / Q000556:secondary / Q000628:therapy; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis; D010052:Ovariectomy; D010220:Para-Aortic Bodies; D010534:Peritoneal Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000556:secondary; D058994:Salpingectomy; D014594:Uterine Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D014599:Uterus / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide
keywords: Ewing sarcoma breakpoint region 1 gene; adult; neuroblastoma; primitive neuroectodermal tumor; uterine neoplasms