Four children, with histopathologically confirmed posterior fossa primitive neuroectoderrnal tumors, were examined by plain radiography, computed tomography (CT) and cerebral angiography. The homogeneously well enhanced solid mass in the midline of the posterior fossa and hydrocephalus of various degree were seen on all CT scans. One case had calcifications and another case had low density areas in the tumor mass. Three cerebral angiograms showed vascular displacement without tumor vascularities. Unfortunately, these CT and angiography findings are and other tumors. when a well enhanced solid mass in the midline posterior fossa is seen on CT scan in children.
Angiography ; Cerebral Angiography ; Child ; Humans ; Hydrocephalus ; Medulloblastoma* ; Neuroectodermal Tumors, Primitive* ; Pathology* ; Radiography ; Tomography, X-Ray Computed

BACKGROUND: 'Eight drugs in a day' was one of the widely used regimen in medulloblastoma. Result of treatment of this regimen and comparison between pre-RT chemotherapy and post-RT chemotherapy were presented. METHODS: Medical records of children who were diagnosed as medulloblastoma and treated with 8 in 1 therapy in Seoul National University Children's Hospital from January 1986 to June 1997 were reviewed. RESULTS: 1) Forty nine cases(male: 30, female: 19) were analyzed. The age at diagnosis was between 3 months and 15 years 3 months and median age was 7 years 10 months. 2) The T stage by Chang classification revealed T1(2%), T2(26%), T3a(9%), T3b(56%), and T4(7%) in 43 cases. M stage revealed M0(46%), M1(15%), M3(37%), and M4(2%) in 41 cases. The surgical results revealed gross total resection(36%), near total resection(18%), subtotal resection(38%), and partial resection(9%). 3) The 5-yr disease free survival(DFS) rate of all tumors was 53%. There was no difference in DFS about sex, age, pathology, T stage, M stage, and surgical result. 4) Difference of 5-year DFS between pre-RT chemotherapy group(53%) and post-RT chemotherapy group(83%) was significant[-2Log(LR), P=0.023], excluding the patient relapsed or too young(below 18 month, who had more chemotherapy to delay radiation) to be received radiation therapy. CONCLUSIONS: DFS in post-RT chemotherapy was better than pre-RT chemotherapy. As medulloblastoma has the property to confine in CNS system, prior treatment with radiation as a intensive local therapy may be more effective than the systemic chemotherapy. Progression or relapse were the main causes of treatment failure. Result of treatment may be improved by more intensive therapy.
Child ; Classification ; Diagnosis ; Drug Therapy* ; Female ; Humans ; Medical Records ; Medulloblastoma* ; Pathology ; Recurrence ; Seoul ; Treatment Failure

Cerebellar Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Follow-Up Studies ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Medulloblastoma ; diagnosis ; pathology ; radiotherapy ; surgery

Adult ; Humans ; Rhabdoid Tumor/surgery* ; Teratoma/pathology* ; Medulloblastoma ; Central Nervous System Neoplasms ; Cerebellar Neoplasms ; Brain Neoplasms/pathology*

Medulloblastoma is a common malignant central nervous system neoplasm found mainly in children. One the contrary, medulloblastoma of the cerebellopontine angle, the location of the tumor is very unusual. This is the the first case of the medullomyoblastoma, a rare form of medulloblastoma, occurring in the cerebellopontine angle. A 15-year-old boy experienced a sudden hearing loss in the left ear. Conservative medical treatment failed, and temporal MR imaging revealed a heterogeneously enhancing mass at the left cerebellopontine angle cistern and in the internal auditory canal; therefore, the lesion was regarded as a typical acoustic neuroma. Few days before surgery, an ipsilateral facial palsy developed, and a follow-up MR imaging showed a rapid growth of the previous lesion. The extended translabyrinthine approach permitted surgical removal. And under pathological diagnosis of malignancy, radiation therapy and series of chemotherapy was performed.
Adolescent ; Cerebellar Neoplasms/*pathology/surgery ; Cerebellopontine Angle/*pathology ; Diagnosis, Differential ; Humans ; Magnetic Resonance Imaging ; Male ; Medulloblastoma/*pathology/surgery ; Neuroma, Acoustic/*pathology

Many advances have been made in understanding the molecular biology of brain tumors through progress in cell and developmental biology. The molecular characterization of rare genetic disorders and tumors shed light on several common tumorigenic mechanisms: mitogenic signaling, cell cycle control, development, and cell surface-cytoskeleton interactions. Discovery of many new molecular markers and the recent identification of brain tumor stem cells offer new directions in future research for tumor molecular diagnostics and therapies.
Brain Neoplasms* ; Brain* ; Cell Cycle Checkpoints ; Developmental Biology ; Glioma ; Medulloblastoma ; Meningioma ; Molecular Biology* ; Pathology, Molecular ; Stem Cells

Cytologic evaluation of cerebrospinal fluid(CSF) is an effective mean for diagnosing many disorders involving the central nervous systems(CNS). One of the most important reasons for cytologic examination of CSF is to detect metastatic or primary neoplasms of the CNS. We did a retrospective study of 1,438 CSF specimens obtained between 1992 and 1996. A total of 1,205 adult and 233 pediatric CSF specimens from 947 patients were accessed at the Department of Pathology of Seoul National University Hospital and Children's Hospital, respectively. Among 1,438 CSF cytology specimens, 169 cases(11.8%, 77 patients) including 135 adult cases(59 patients) and 34 pediatric cases(18 patients) were positive for malignant cells. Diagnoses included 60 metastatic carcinomas(adult, 60; pediatric, 0); 46 malignant lymphomas(adult, 44; pediatric, 2); 21 leukemias(adult, 20; pediatric, 1); 4 retinoblastomas(adult, 0; pediatric 4); 2 rhabdomyosarcomas(adult, 0; pediatric, 2); 1 multiple myeloma(adult, 1; pediatric, 0), and 35 primary CNS neoplasms(adult, 10; pediatric, 25). The most commonly identified metastatic carcinomas in adults were adenocarcinoma. Their primary sites were the lung, gastrointestinal tract, and breast in order of frequency. The most common primary CNS neoplasm in children was medulloblastoma.
Adenocarcinoma ; Adult ; Breast ; Central Nervous System ; Cerebrospinal Fluid* ; Child ; Diagnosis ; Gastrointestinal Tract ; Humans ; Lung ; Medulloblastoma ; Pathology ; Retrospective Studies ; Seoul

OBJECTIVETo investigate the apoptosis and proliferation effect of matrine on human medulloblastoma cell line D341 in vitro and the effect of the expression of the related caspase 3 and caspase 9 proteins.

METHODSThe D341 cells were cultivated successfully in vitro. Then the cells were divided into 5 groups according to the concentration of matrine (0.5 mg/mI group, 1.0 mg/ml group, 1.5 mg/ml group, 2.0 mg/ml group and the control group was 0 mg/ml). All the experiments were repeated three times. The cell morphologic and structure change was observed with the optical microscope and the transmission electron microscope. The proliferation of D341 cell was analyzed using Cell Counting Kit-8 assay. Apoptosis was detected by Annexin V-FITC/PI double staining. The expression of Caspase3 and Caspase9 was detected by Western blot.

RESULTSWith the effect of matrine, the proliferation inhibition rate gradually increased with drug concentrations increasing, and there was a significant difference (P < 0.01). The inhibitory effect of matrine on cell proliferation was different with the different treatment time, there was a significant difference between the 24 h to 72 h groups (P < 0.01). The apoptotic rate increased with matrine concentrations increasing. There were significant differences between the group of 0.5 mg/mI or 1.0 mg/mI to the group of 1.5 mg/mI or 2.0 mg/mI (P < 0.05). The apoptotic rate increased with the prolonged treatment time. There were significant differences between the group of 24 h or 48 h to the group of 72 h ( P < 0.05). With the increase of matrine concentration, the expression of Caspase 3 and Caspase 9 increased (P < 0.01).

CONCLUSIONMatrine induces the apoptosis, and inhibits the proliferation of human medulloblastoma D341 cells in vitro by up-regulation of the expression level of Caspase3, Caspase9.

Alkaloids ; pharmacology ; Apoptosis ; Caspase 3 ; metabolism ; Caspase 9 ; metabolism ; Cell Line, Tumor ; Cell Proliferation ; Cerebellar Neoplasms ; metabolism ; pathology ; Humans ; Medulloblastoma ; metabolism ; pathology ; Quinolizines ; pharmacology ; Up-Regulation

OBJECTIVETo study the clinicopathologic features, immunohistochemical findings, diagnosis and differential diagnosis of atypical teratoid/rhabdoid tumors (AT/RT) of central nervous system in childhood.

METHODSThe clinicopathologic data, morphologic features and immunophenotypes were reviewed in 6 cases of AT/RT. EnVision method was applied. Antibodies include cytokeratin (CK), epithelial membrane antigen (EMA), vimentin, smooth muscle actin (SMA), muscle specific actin (MSA), glial fibrinary acid protein (GFAP), desmin, placental alkaline phosphatase (PLAP) and INI1.

RESULTSFive of the six cases of AT/RT occurred in infancy and early childhood. Histologically, the predominant component was rhabdoid cells. Cytoplasmic inclusions were present in all cases. Primitive neuroectodermal tumor (PNET) component was also identified in 5 of the 6 cases studied. Immunohistochemically, the tumor cells were positive for cytokeratin, epithelial membrane antigen and vimentin. The staining for INI1, desmin and PLAP was negative. Smooth muscle actin was expressed in 2 cases and glial fibrillary acidic protein in 5 cases. The proliferative index as demonstrated by Ki-67 staining was high.

CONCLUSIONSAT/RT is not a particularly uncommon malignancy in childhood. The histologic hallmark is the presence of rhabdoid cells with cytoplasmic inclusions. The tumor cells are positive for cytokeratin, epithelial membrane antigen and vimentin, and negative for INI1. Differential diagnosis includes PNET, medulloblastoma and medullomyoblastoma.

Brain Neoplasms ; metabolism ; pathology ; surgery ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Keratins ; metabolism ; Male ; Medulloblastoma ; metabolism ; pathology ; Mucin-1 ; metabolism ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Rhabdoid Tumor ; metabolism ; pathology ; surgery ; Teratoma ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

The posterior fossa, which contains about one fourth of the intracranial contents, is the site of known about two thirds of pediatric intracranial tumors. In about such tumors are less common and the prognosis may bevery poor. However, it is important to recognize the earliest symptoms because of the relatively high ease rate of operable management and the possibility for a high cure rate in some cases. A series of 51 pathologically verified posterior fossa tumors seen at the Department of Neurosurgery, Yonsei University, Severance Hospital over several years were studied. These tumors were explored surgically, and the diagnosis being made through histological examination. The cases were reviewed as to history, development and progression of the neurological features, laboratory findings, roentgenograms, differential diagnosis, operative methods and findings, and pathology. The authors emphasized some interesting points which are illustrated as follows; 1. Among 51 cases of posterior fossa tumors, 25 cases of acoustic neuroma, 9 cases of astrocytoma, 9 cases of medulloblastoma, 2 cases of ependymoma, 3 cases of pontine glioma and 3 cases of cysticercosis in the fourth ventricle were found, comprising 21 males and 30 females. 2. The 25 operated cases of acoustic neuroma were between the age of 20 and 60. Eighteen cases(69.2%) of the remaining posterior fossa tumors were below the age of 15. 3. The author was able to make the correct plain film diagnosis of acoustic neuroma in 13 cases(51%) of 25 cases. The prominent angiographic and ventriculographic finding of posterior fossa tumors was ventricular dilatation. 4. Vntriculogram was very important in the diagnosis of the fourth ventricle tumors. The Conray ventriculogram had the special benefit in accurately outlining and demonstrating the invasive pattern of fourth ventricle tumors. The Conray showed C.S.F. flow in malignant and other large tumors in the fourth ventricle better than was shown by previous pneumo-ventriculograms. 5. The Electroencephalogram showed an abnormal pattern in 62.2 per cent of 29 posterior fossa tumors.
Astrocytoma ; Cysticercosis ; Diagnosis ; Diagnosis, Differential ; Dilatation ; Electroencephalography ; Ependymoma ; Female ; Fourth Ventricle ; Glioma ; Humans ; Infratentorial Neoplasms* ; Male ; Medulloblastoma ; Neuroma, Acoustic ; Neurosurgery ; Pathology ; Prognosis