Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.
Adult ; Carcinoma, Papillary/*pathology/surgery ; Case Report ; Diagnosis, Differential ; Ependymoma/*pathology/surgery ; Fatal Outcome ; Female ; Follow-Up Studies ; Glioma/*pathology ; Human ; Magnetic Resonance Imaging ; Middle Age

Adolescent ; Brain Neoplasms ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Ganglioglioma ; metabolism ; pathology ; surgery ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Male ; Tubulin ; metabolism ; Vimentin ; metabolism

Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Female ; Hemangioblastoma ; metabolism ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Meningeal Neoplasms ; metabolism ; pathology ; surgery ; Meningioma ; metabolism ; pathology ; surgery ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Vimentin ; metabolism

Astrocytoma ; metabolism ; pathology ; surgery ; Brain Neoplasms ; metabolism ; pathology ; surgery ; Child, Preschool ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neurofibroma, Plexiform ; metabolism ; pathology ; S100 Proteins ; metabolism ; Vimentin ; metabolism

Antigens, Nuclear ; metabolism ; Cordotomy ; methods ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Nerve Tissue Proteins ; metabolism ; Neurocytoma ; metabolism ; pathology ; surgery ; Oligodendroglioma ; S100 Proteins ; metabolism ; Spinal Cord Neoplasms ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism

Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Central Nervous System Neoplasms ; metabolism ; pathology ; surgery ; ultrastructure ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Hemangioblastoma ; metabolism ; pathology ; surgery ; ultrastructure ; Humans ; Meningeal Neoplasms ; metabolism ; pathology ; Meningioma ; metabolism ; pathology ; Mutation ; Neoplasm Recurrence, Local ; Prognosis ; Receptor, Epidermal Growth Factor ; metabolism ; Vimentin ; metabolism ; von Hippel-Lindau Disease ; genetics ; metabolism ; pathology ; surgery

Adolescent ; Adult ; Aged ; Diagnosis, Differential ; Ependymoma ; pathology ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Meningeal Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Meningioma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Mucin-1 ; metabolism ; Oligodendroglioma ; pathology ; Tomography, X-Ray Computed ; Vimentin ; metabolism ; Young Adult

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Cystadenocarcinoma, Papillary ; pathology ; Dactinomycin ; therapeutic use ; Diagnosis, Differential ; Ependymoma ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Hysterectomy ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Ovariectomy ; Teratoma ; pathology ; Vimentin ; metabolism ; Vincristine ; therapeutic use

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