Child ; Family ; Humans ; Neuroectodermal Tumors, Primitive/pathology* ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Oropharynx

Ewing's sarcoma in the cervix is characterized by extremely rare occurrence,high degree of malignancy,and rapid progression.The diagnosis of this disease is based on pathology and immunohistochemistry. The main image of the case reported in this paper showed the cervical cyst with solid mass,large volume,and uneven density and signal,and the solid part can be strengthened in enhanced scanning.Because of the rapid growth,the lesion is prone to liquefaction necrosis and bleeding.Since the metastasis occurs early,timely diagnosis is essential.
Cervix Uteri/pathology* ; Female ; Humans ; Immunohistochemistry ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Sarcoma, Ewing/pathology* ; Uterine Cervical Neoplasms

OBJECTIVETo study clinicopathologic features, immunohistochemical profile, diagnosis and differential diagnosis of childhood central nervous system primitive neuroectodermal tumors (CNS PNETs) with the features of ependymoblastoma and neuroblastoma.

METHODSThe clinical data, morphologic and immunohistochemical features were analyzed in 4 cases of pediatric CNS PNETs with features of ependymoblastoma and neuroblastoma. EnVision method immunohistochemistry was applied.

RESULTSFour patients including three boys and one girl presented at the age from 12 month to 4 years and three tumors located in cerebrum, one in brain stem. All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils. There was focal expression of glial fibrillary acidic protein (GFAP) consistent with glial differentiation and epithelial membrane antigen (EMA) consistent with ependymal differentiation. Necrosis was seen in three cases and calcification was present in one case. Immunohistochemically, the rosettes and undifferentiated neuroepithelial cells were positive for vimentin, partially positive for GFAP and EMA but negative for synaptophysin. The tumor cells were also positive for synaptophysin in neuropils. The Ki-67 label index ranged from 20% to 60%.

CONCLUSIONSCNS PNETs with the features of ependymoblastoma and neuroblastoma is a rare tumor with poor prognosis. The tumor primarily occurs in childhood, especially infant and belongs to the family of embryonal tumors of the CNS. The morphologic, immunohistochemical and genetic features are important in differential diagnosis from other tumors of the CNS.

Antigens, Neoplasm ; metabolism ; Central Nervous System ; pathology ; Child ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Male ; Mucin-1 ; metabolism ; Neuroblastoma ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Vimentin ; metabolism

12E7 Antigen ; Adolescent ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Lymphoma ; metabolism ; pathology ; Male ; Neoplastic Cells, Circulating ; Nephrectomy ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Venae Cavae ; pathology ; Vimentin ; metabolism ; Wilms Tumor ; metabolism ; pathology

OBJECTIVETo compare the pathologic diagnosis and immunohistochemistry of small cell malignant tumors (SCMT) of bone using both core needle biopsy and surgical specimen.

METHODSSeventy-seven cases of SCMT with core needle biopsies and surgical specimens available were respectively analyzed by histologic examination and immunohistochemical study, with literature review.

RESULTSThe male-to-female ratio was 48:29. The age of the patients ranged from 6 to 73 years. The tumors studied included Ewing sarcoma/PNET (n = 38), myeloma (n = 23), lymphoma (n = 10), small cell osteosarcoma (n = 2), small cell carcinoma (n = 2) and mesenchymal chondrosarcoma (n = 2). The tumors involved limbs, axial skeleton and flat bones. Microscopically, the tumors shared similar histology, with small round cells and spindly cells arranged in diffuse sheets. The pathologic diagnosis by core needle biopsies correlated with that by surgical specimens in 84.4% (65/77) of the cases.

CONCLUSIONSSCMT represents a heterogeneous group of malignancy. Correlations with clinicoradiologic findings and application of ancillary investigations including immunohistochemistry and molecular study are important for definitive diagnosis. Pathologic diagnosis using core needle biopsies shows good results and provides useful information for surgical planning.

12E7 Antigen ; Adolescent ; Adult ; Aged ; Antigens, CD ; metabolism ; Biopsy, Large-Core Needle ; Bone Neoplasms ; diagnosis ; metabolism ; pathology ; Carcinoma, Small Cell ; diagnosis ; metabolism ; pathology ; Cell Adhesion Molecules ; metabolism ; Child ; Female ; Humans ; Lymphoma ; diagnosis ; metabolism ; pathology ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; metabolism ; pathology ; Oncogene Proteins, Fusion ; metabolism ; Osteosarcoma ; diagnosis ; metabolism ; pathology ; Plasmacytoma ; diagnosis ; metabolism ; pathology ; Proto-Oncogene Protein c-fli-1 ; metabolism ; RNA-Binding Protein EWS ; metabolism ; Retrospective Studies ; Sarcoma, Ewing ; diagnosis ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic features of primitive neuroectodermal tumor (PNET) in female genital tract.

METHODSSix cases of PNET arising in female genital tract were retrospectively reviewed. The clinicopathologic features, immunohistochemical findings and EWS gene translocation study results were analyzed.

RESULTSThe age of patients ranged from 10 to 27 years (mean = 20 years). The sites of involvement included ovary (1 case), uterus (1 case), vulva (2 cases) and vagina (2 cases). The greatest diameter of the tumor ranged from 2 to 10 cm (mean = 5.4 cm). The tumor had nodular appearance and showed grayish-pink fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Histologically, the tumor was composed of malignant small round cells with indistinct cell borders, hyperchromatic nuclei, dense chromatin, tiny nucleoli and scanty cytoplasm. The tumor cells were arranged in sheets or lobules. Homer-Wright rosettes were identified in 1 case. Immunohistochemical study showed that the tumor cells were positive for CD99, FLI-1 and CD56 (6/6). Focal expression of vimentin (5/6), NSE (5/6), nestin (4/6), synaptophysin (4/6), S-100 protein (2/6) and chromogranin A (1/6) was also demonstrated. EWS gene translocation was detected in 5 cases studied. Follow-up information was available in 2 patients (7 and 17 months of follow up, respectively). One of them died of tumor metastasis 17 months after diagnosis. The other patient was still alive.

CONCLUSIONSPNET arising in female genital tract is rare. It mainly involves ovary, uterus, vulva and vagina. Immunohistochemical study using a panel of antibodies and fluorescence in-situ hybridization play an important role in definitive diagnosis of this rare malignancy.

12E7 Antigen ; Adolescent ; Adult ; Antigens, CD ; metabolism ; CD56 Antigen ; metabolism ; Cell Adhesion Molecules ; metabolism ; Child ; Female ; Follow-Up Studies ; Genital Neoplasms, Female ; genetics ; metabolism ; pathology ; surgery ; Humans ; Neuroectodermal Tumors, Primitive, Peripheral ; genetics ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Proto-Oncogene Protein c-fli-1 ; metabolism ; RNA-Binding Protein EWS ; genetics ; Retrospective Studies ; Translocation, Genetic ; Uterine Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vaginal Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Vulvar Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Young Adult

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an uncommon, aggressive, and malignant tumor with a poor patient outcome. Its occurrence in the lesser sac is a rare event and to the best of our knowledge, has not been previously described. The present case was clinically and radiologically misdiagnosed as a pancreatic tumor/gastrointestinal stromal tumor. Histopathology revealed a tumor with "small round cells" that were positive for CD99, confirming the diagnosis of ES/PNET. This report highlights the importance of considering Ewing's sarcoma in the differential diagnosis of intraabdominal, extraintestinal masses.
Antigens, CD/analysis ; Biopsy ; Cell Adhesion Molecules/analysis ; *Diagnostic Errors ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral/*diagnosis/immunology/pathology/therapy ; Pancreatic Neoplasms/*diagnosis ; Peritoneal Neoplasms/*diagnosis/immunology/pathology/therapy ; Predictive Value of Tests ; Sarcoma, Ewing/*diagnosis/immunology/pathology/therapy ; Tomography, X-Ray Computed ; Tumor Markers, Biological/analysis

Fatal Outcome ; Humans ; Magnetic Resonance Imaging ; Male ; Mediastinal Neoplasms ; complications ; diagnostic imaging ; pathology ; surgery ; Mediastinum ; diagnostic imaging ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; complications ; diagnostic imaging ; pathology ; surgery ; Radiography ; Spinal Cord Diseases ; diagnostic imaging ; etiology ; pathology ; Young Adult

OBJECTIVETo investigate the efficacy of high dose chemotherapy combined with autologous peripheral blood stem cell transplantation (APBSCT) for the treatment of neural ectodermal solid tumor originated from neural crest in children.

METHODSTwenty-three children at a medium age of 5.8 + or - 3.5 years with neural ectodermal solid tumor originated from neural crest were enrolled. Of the 23 children, 20 with stage IV neuroblastoma (9 were in complete remission, 7 were in partial remission and 4 were in progressive disease), 2 with stage IV primitive neuroectodermal tumor (PNET) in complete remission, and 1 with retinoblastoma in partial remission. Before APBSCT the children received 8.0 + or - 4.3 courses of chemotherapy. During chemotherapy the autologous peripheral blood stem cells were harvested and the tumor excision was performed. Then APBSCT was performed.

RESULTSThe reconstruction of the hematopoietic system was noted in 19 of 20 children with stage IV neuroblastoma 16.5 + or - 0.9 days after transplantation. A follow-up (median 15.8 months) was done in these children. The follow-up showed that the survival rate in children in complete remission before transplantation was 100%, 57% in those in partial remission, and none of children in progressive disease survived (P<0.05). The total survival rate was 67% in children with neuroblastoma. The child with retinoblastoma had complete remission in a 6-months follow-up. The tumors recurred in children with PNET 5 to 8 months after transplantation and all died within one year after transplantation.

CONCLUSIONSHigh dose chemotherapy combined with APBSCT can result in a good outcome in children with neural ectodermal solid tumor originated from neural crest in complete remission before transplantation and can improve the outcome in patients in partial remission before transplantation. However, the children with PNET, even in complete remission before transplantation, do not respond to the therapy.

Antigens, CD34 ; analysis ; Antineoplastic Agents ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Neural Crest ; pathology ; Neuroblastoma ; therapy ; Neuroectodermal Tumors, Primitive ; therapy ; Peripheral Blood Stem Cell Transplantation ; Transplantation, Autologous

OBJECTIVETo observe the expression of FLI-1 in primitive neuroectodermal tumors (PNET), explore the value of immunohistochemical staining of FLI-1 in combination with other neural markers in diagnosis of PNET, and analyze the prognostic factors in PNET patients.

METHODS35 cases of PNET, of which 33 cases with complete clinical data, were included in this study. Immmunohistochemistry (The En Vision method) was applied to detect the expression of FLI-1, CD99, Syn, NSE, S-100, NF, Vim in the tumor tissues. The clinicopathological data of 33 cases were analyzed by Cox regression.

RESULTSThe positive expression rate of FLI-1 were 51.4% and that of CD99 was 88.6%. The sensitivity of FLI-1 combined with CD99 was up to 100%. The positive rates of Vim, Syn, NSE, s-100 and NF were 91.4%, 48.6%, 45.7%, 22.9% and 0, respectively. Cox regression analysis showed that the impact of primary location and treatment modality were of statistical significance (P < 0.05), but the age, sex, stage or size of tumors did not (P > 0.05).

CONCLUSIONImmunohistochemical detection of FLI-1 and neural markers is a preferred method for clinical diagnosis of PNET. The main factors affecting the prognosis are the primary location of PNET and treatment modality.

12E7 Antigen ; Adolescent ; Adult ; Antigens, CD ; metabolism ; Brain Neoplasms ; metabolism ; pathology ; therapy ; Cell Adhesion Molecules ; metabolism ; Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; therapy ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; therapy ; Pelvic Neoplasms ; metabolism ; pathology ; therapy ; Phosphopyruvate Hydratase ; metabolism ; Proportional Hazards Models ; Proto-Oncogene Protein c-fli-1 ; metabolism ; S100 Proteins ; metabolism ; Survival Rate ; Synaptophysin ; metabolism ; Vimentin ; metabolism ; Young Adult