Child ; Female ; Humans ; Lumbar Vertebrae ; Neuroectodermal Tumors, Primitive ; pathology ; therapy ; Spinal Neoplasms ; pathology ; therapy

A primary intraspinal primitive neuroectodermal tumor is very rare, with only 24 cases having been reported in the literature. In general this type of tumor is treated with surgery followed by radiotherapy and chemotherapy; however, the prognosis still remains poor. The case of a primary intraspinal primitive neuroectodermal tumor, at the conus medullaris in a 17 year old male patient is presented. He had sufferred from paraparesis, urinary difficulty and lower back pain of 1 month duration. A thoracolumbar MRI demonstrated a 2x2x8cm isointense intraspinal mass, on T1-weighted images, with strong contrast enhancement from the T11 to L2 level. There was no clinical or radiological evidence for the existence of an intracranial tumor. A histological examination revealed a small round cell tumor and immunohistochemical characteristics of PNET. The clinical, radiological and pathological features are discussed with a review of the literatures.
Adolescent ; Human ; Lumbar Vertebrae ; Magnetic Resonance Imaging ; Male ; Neuroectodermal Tumors, Primitive/*pathology/therapy ; Spinal Cord Neoplasms/*pathology/therapy ; Thoracic Vertebrae

OBJECTIVETo review the clinical characteristics, diagnosis and treatments of primitive neuroectodermal tumor (PNET) in head and neck.

METHODSA retrospective review of the medical records for the cases of PNET in head and neck from 2004 to 2014. General clinical information including diagnosis and treatments was obtained and analyzed. A literature review was also conducted.

RESULTSA total of 5 cases diagnosed with PNET were included. Of 5 patients, one patient with lesion in the temporal field was treated with radical resection, followed by radiotherapy, and a 24-month follow-up showed no recurrence. Three patients presented with maxillary, infratemporal fossa or cheek invasion respectively, after diagnosed with pathological examination, one patient received preoperation radiotherapy plus radical resection and postoperative chemotherapy, and the lesions had no obvious progress with follow-up of 106 months; one patient was applied with preoperative chemotherapy plus extensive resection and postoperation radiotherapy, showed recurrence 15 months later, and was lost to follow-up; and another patient underwent chemotherapy plus radiotherapy and extensive resection, presented with pulmonary metastasis 5 months later, and died of brain metastasis within 25 months. One foreign patient presented with tumor involved submaxillary, mouth and tongue, the tumor was reduced obviously after chemotherapy, but he was lost to follow-up after getting home.

CONCLUSIONSPNET in head and neck is rare and the clinical profile of PNET is the presence of occupying and compression. The pathology examination is an only way to confirm the diagnosis of PNET. The combination of excision, radiotherapy and chemotherapy is the treatment choice.

Brain Neoplasms ; secondary ; Head and Neck Neoplasms ; diagnosis ; pathology ; therapy ; Humans ; Lost to Follow-Up ; Male ; Neoplasm Recurrence, Local ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; therapy ; Retrospective Studies

OBJECTIVE: Strategies for managing supratentorial primitive neuroectodermal tumors(PNETs) in children include surgical resection, craniospinal irradiation and chemotherapy. This study is performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. METHODS: We have reviewed all medical records and pathology slides of six children(four males and two females) with supratentorial PNET from November, 1987 to May, 2003. The extent of resection was confirmed by computed tomography and magnetic resonance studies. RESULTS: The patients were aged 1 to 13 years and treated postoperatively with/without adjuvant therapy. Tumor location included was four cortical, one gangliobasal, and one pineal region. The presenting symptoms and signs consisted of increased intracranial pressure and focal neurological deficits such as seizure and hemiparesis. The treatment consisted of surgical resection alone in one patient, postoperative radiotherapy in one patient, postoperative chemotherapy in one, and postoperative radiotherapy with chemotherapy in three. Five patients lived more than 12 months after diagnosis and one patient among them has been living more than 5 years after diagnosis. CONCLUSION: We can improve the survival and prognosis of supratentorial PNET patients by radical gross total resection of tumor followed by craniospinal irradiation and aggressive chemotherapy. First of all, gross total resection of tumor is the most important among many factors.
Child* ; Craniospinal Irradiation ; Diagnosis ; Drug Therapy ; Humans ; Intracranial Pressure ; Male ; Medical Records ; Neural Plate ; Neuroectodermal Tumors, Primitive* ; Paresis ; Pathology ; Prognosis ; Radiotherapy ; Seizures

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

12E7 Antigen ; Antigens, CD ; metabolism ; CD57 Antigens ; metabolism ; Cell Adhesion Molecules ; metabolism ; Female ; Humans ; Immunohistochemistry ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; therapy ; Pancreas ; chemistry ; pathology ; surgery ; Pancreatectomy ; methods ; Pancreatic Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; 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

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

Adult ; Antineoplastic Agents ; therapeutic use ; Benzenesulfonates ; therapeutic use ; Female ; Humans ; Kidney Neoplasms ; diagnostic imaging ; drug therapy ; Neuroectodermal Tumors, Primitive ; diagnostic imaging ; drug therapy ; Niacinamide ; analogs & derivatives ; Phenylurea Compounds ; Pregnancy ; Pyridines ; therapeutic use ; Radiography ; Thrombosis ; diagnostic imaging ; pathology ; Vena Cava, Inferior ; diagnostic imaging ; pathology

Carcinoma ; drug therapy ; genetics ; metabolism ; pathology ; radiotherapy ; Desmoplastic Small Round Cell Tumor ; metabolism ; pathology ; Diagnosis, Differential ; Gene Rearrangement ; Head and Neck Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Humans ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Lymphatic Metastasis ; Male ; Mediastinal Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Melanoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Nuclear Proteins ; genetics ; metabolism ; Oncogene Proteins ; genetics ; metabolism ; Oncogene Proteins, Fusion ; genetics ; metabolism ; Rhabdomyosarcoma ; metabolism ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy