Dysembryoplastic neuroepithelial tumors (DNETs) arise mostly in the supratentorial cerebral cortex. A very rare case of intraventricular DNET with diffuse ependymal involvement, which causes spinal drop metastasis, is presented.
Adult ; Diagnosis, Differential ; Ependymoma/*pathology/radiotherapy ; Female ; Humans ; Lumbosacral Region/*pathology ; *Magnetic Resonance Imaging ; Neuroepithelial Cells/pathology ; Spinal Neoplasms/radiotherapy/*secondary

BACKGROUND: The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. METHODS: Between 1997 and 2013, 33 patients with 25 ependymomas (WHO grade II) and eight anaplastic ependymomas (WHO grade III) were pathologically diagnosed. Six were pediatric patients (mean age, 6.15 years; range, 1.3–11 years), while 27 were adults (mean age, 47.5 years; range, 19–70 years). Of those, there were 12 adult patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment. Prognostic factors were assessed in ependymoma patients. Prognostic factors were studied using Kaplan-Meier estimates in subgroups. RESULTS: For six pediatric patients, the progression-free survival (PFS) was 43.7±13.5 months, and the overall survival (OS) was 58.1±13.7 months. For 27 adult patients, the PFS was 125.6±14.3 months, and the OS was 151.2±12.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). Out of 12 patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment, one patient showed tumor recurrence during follow-up (mean, 93.5 months; range, 27.9–162.7 months). CONCLUSION: Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
Adult ; Disease-Free Survival ; Ependymoma* ; Follow-Up Studies ; Humans ; Pathology ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies* ; Survival Rate

OBJECTIVETo study the clinicopathologic features of papillary tumor of the pineal region (PTPR).

METHODThree hundred and eighty six cases of pineal region and posterior third ventricle tumors, two newborn and two adult pineal glands were analyzed by HE, PAS and immunohistochemistry of 16 antibodies (EnVision method).

RESULTSFive cases of PTPR were diagnosed with mixed papillary features and densely cellular areas, and included one recurrent case. In the papillary areas, the vessels were lined by one or several layers of cuboidal/columnar cells; the vessel wall was hyalinized. In the densely cellular areas, sheets or nests of tumor cells were seen. The tumor cells of these five cases were immunoreactive to CK, CK8/18, synaptophysin, MAP2, nestin, S-100, and vimentin. Four cases were immunoreactive to NSE and CgA; and 2 cases were immunoreactive to NF. All five cases were negative for EMA, CK5/6, CEA, and NeuN. Ki-67 labeling index ranged from 1% to 6%.Three patients were alive, and the recurrent one died.

CONCLUSIONSPTPR occurs in patients with over a wide age range, from children to adults, and is more commonly found in male than female. PTPR is composed of both papillary and solid areas, characterized by epithelial cytology, and needs to be differentiated from ependymoma. PTPR may originate from the specialized ependymocytes of the subcommissural organ. The prognostic factors are early diagnosis, complete surgical resection and radiotherapy.

Adolescent ; Adult ; Brain Neoplasms ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery ; Carcinoma, Papillary ; diagnostic imaging ; metabolism ; pathology ; radiotherapy ; surgery ; Child ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Keratin-18 ; metabolism ; Keratin-8 ; metabolism ; Keratins ; metabolism ; Male ; Microtubule-Associated Proteins ; metabolism ; Nestin ; metabolism ; Pineal Gland ; Pinealoma ; metabolism ; pathology ; S100 Proteins ; metabolism ; Synaptophysin ; metabolism ; Tomography, X-Ray Computed ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo investigate the diagnostic method and analyze the result of microneurosurgical treatment for tumors of the fourth cerebral ventricle.

METHODSTumor of the fourth ventricle was clinically diagnosed in 86 patients basing on the preliminary assessment of symptom and CT or MRI findings. Of these 86 patients treated with micro-neurosurgery, the tumors in 62 were totally removed, subtotally in 19, and partially in 5. Forty-two patients received postoperative radiotherapy.

RESULTSThree patients died postoperatively within ten days, and symptoms in 83 were improved after treatment. The average survival period was over 3 years. The pathology included 32 medulloblastomas, 23 ependymoma, 15 astrocytoma, 10 hemangiblastomas, 2 choroid plexus papillomas, and 4 epidermoid cysts.

CONCLUSIONMedulloblastoma, astrocytoma and hemangiblastoma are suggested to be removed totally whenever technically possible according to the site, character and volume of the tumor. For ependymoma, if close to the brain stem, is recommended to be subtotally removed. Postoperative radiotherapy may be beneficial for malignant types.

Adolescent ; Adult ; Aged ; Astrocytoma ; diagnosis ; diagnostic imaging ; surgery ; Cerebral Ventricle Neoplasms ; diagnosis ; radiotherapy ; surgery ; Child ; Child, Preschool ; Combined Modality Therapy ; Ependymoma ; diagnosis ; diagnostic imaging ; surgery ; Female ; Follow-Up Studies ; Fourth Ventricle ; pathology ; radiation effects ; surgery ; Hemangioblastoma ; diagnosis ; diagnostic imaging ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Medulloblastoma ; diagnosis ; diagnostic imaging ; surgery ; Microsurgery ; methods ; mortality ; Middle Aged ; Neoplasm Recurrence, Local ; Survival Analysis ; Survival Rate ; Tomography, X-Ray Computed