Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome.

Benign notochordal cell tumours have recently been described as intraosseous benign lesions of notochordal cell origin. The lesions are found in vertebral bodies in 20% of autopsy studies and are a potential precursor of chordoma. We report a rare case of lumbar vertebral chordoma that was thought to arise from a benign intraosseous notochordal cell tumour and which showed significant osteosclerotic change. Radiologically, the lumbar vertebral mass lesion showed hyperintensity on T2 weighted images, with scanty enhancement on post-contrast T1 weighted MR images. High uptake corresponding to the mass was noted on fluorine-18-fluorodeoxyglucose positron emission tomography. Bone biopsy revealed proliferation of the physaliphorous cells between thickened bone trabeculae; no nuclear mitosis was observed. Although the mass was diagnosed clinically as spinal chordoma, histopathology contained both benign notochordal cell tumour and conventional chordoma. After heavy particle (11C)-charged radiation therapy was applied to the lesion with a sufficient radiation field margin, the tumour volume significantly decreased and there was improvement in the patient's symptoms. On follow-up radiological studies, the tumour had markedly regressed and there was no tumour regrowth or distant metastasis. In this case report, benign notochordal cell tumour and conventional chordoma are histopathologically identified in the L1 vertebral body, which contains osteosclerotic and osteolytic areas. It is suggested that the benign notochordal cell tumour coexists with a conventional chordoma and that this histopathological finding supports a hypothetical relationship between benign notochordal cell tumour and chordoma.

A case of synovial sarcoma in the perivertebral space of the neck: clinical presentation, radiological findings and histopathological description.

We report a case of synovial sarcoma in the neck that was initially considered to be a benign soft-tissue tumour. The mass appeared to originate in the perivertebral space, extending to the spinal canal via the C3/4 and C4/5 neural foramina. The mass also encased the right vertebral artery and this suggested a malignant soft-tissue tumour.

Expansile organized maxillary sinus hematoma: MR and CT findings and review of literature.

An organized hematoma is a chronic state of fibrotic tissue surrounding a hemorrhage. A mass lesion resulting from hematoma in the maxillary sinus was first reported in 1917, and the term, "blood boil," was clinically coined from such features as encapsulated blood and locally aggressive behavior. Subsequently, others have reported lesions with a similar appearance and clinical course, and now, in Japan, blood boil is used as a clinical term for such lesions. Factors that may predispose a patient to hematoma formation vary, and the pathogenesis of the mass is still uncertain. The lesions are mainly composed of an organized hematoma, regardless of their origin. We present 2 cases of organized maxillary sinus hematomas that have unusual radiologic findings and correlate these findings with the histopathologic findings.

Clinical results of stenting for cervical internal carotid stenoses.

SUMMARY: Total 89 patients with cervical ICA stenosis were treated by stenting. In 74 cases of stenting, we used our blocking balloon systems to prevent distal embolism. The morbidity and the mortality rate was 4.5% and 0%, respectively.Two(3%) of 74 cases showed distal embolism when blocking balloon catheter systems(BBCS) were used, while distal embolism occurred in four (27%) of 15 cases of stenting without BBCS. On diffusion- weighted MRI (DWI), hyperintense areas were detected in seven (47%) of 15 lesions when we used BBCS only during postdilatation. On the other hand, use of BBCS during predilatation as well as postdilatation reduced hyperintense areas on DWI, which were detected in three (25%) of 12 patients. Our blocking balloon catheter system is a useful device to reduce the risk of distal embolism, especially when we use it during not only postdilatation but predilatation.

A case of glassy cell carcinoma of the uterine cervix effectively responding to chemotherapy with paclitaxel and carboplatin.

Glassy cell carcinoma (GCC) of the uterine cervix is a highly malignant tumor and has a poor prognosis. As yet, no effective systemic chemotherapy to this tumor has been reported. Here we describe a case of recurrent GCC that responded to paclitaxel and carboplatin combination treatment. The patient, a 32-year-old woman, with clinical staging FIGO IB1 disease had a radical hysterectomy and postoperative radiotherapy. Three months after initial treatment, she had a relapse as peritoneal dissemination, which was confirmed in the second surgery (adnectomy) and which did not respond to platinum-based conventional chemotherapy (cisplatin, adriamycin, cyclophosphamide and carboplatin, etoposide). The recurrent peritoneal tumors responded well to paclitaxel and carboplatin combination treatment. An elevated serum concentration of carcinoembryonic antigen (672 ng/ml) was reduced to 14.4 ng/ml by six such courses. Peritoneal histopathology confirmed a complete response in the third surgery (ileostomy) for adhesive ileus by the radiotherapy. This is the first report of effective systemic chemotherapy with paclitaxel and carboplatin to recurrent GCC of the uterine cervix.

Variation of des-gamma-carboxyprothrombin (PIVKA-II) levels in cord blood throughout gestation.

The variation of des-gamma-carboxyprothrombin (PIVKA-II, protein induced by vitamin K absence) levels in umbilical cord blood throughout gestation was examined using a highly sensitive method, electrochemiluminescence immunoassay (ECLIA). PIVKA-II levels in infants without any complications were low, but modestly high, exceeding the normal range of healthy adults during the preterm period, followed by a remarkable increase after the 37th week of gestation. Among infants complicated with severe preeclampsia a marked increase of PIVKA-II levels was observed in preterm infants, showing a good correlation with the existence ofinfarctions on the placenta. On the other hand, among infants complicated with preterm premature rupture of the membranes (PROM) in which antibiotics were administered during the prenatal period, a moderate elevation of PIVKA-II levels was observed. These data suggest that the normal range of PIVKA-II in fetuses is modestly high compared with adults and any deficient status of vitamin K would not exist throughout the preterm period. Nevertheless, the vitamin K status might readily fall into a deficient condition in term infants. Furthermore, it is notable that vitamin K deficiency would be induced in complicated gestation with severe preeclampsia and medication with antibiotics.

Urocortin in human placenta and maternal plasma.

Plasma immunoreactive (IR-) urocortin (Ucn) and corticotropin-releasing factor (CRF) levels in pregnant women were measured by their specific radioimmunoassays after extraction. Although plasma IR-CRF levels were increased in pregnant women as compared to men and non-pregnant women, there was no difference of plasma IR-Ucn levels among groups. Ucn mRNA was detected in cytotrophoblasts and syncytiotrophoblasts by in situ hybridization. A reverse-phase high-performance liquid chromatography (HPLC) showed the major peak of IR-Ucn in placenta and plasma that had similar chromatographic mobility to synthetic Ucn1-40. These data suggest that Ucn is produced and processed into the same form of synthetic Ucn in placenta, but not secreted into maternal blood.

Rel transcription factors contribute to elevated urokinase expression in human ovarian carcinoma cells.

Elevated levels of the urokinase-type plasminogen activator (uPA) in tumor cells are conductive to tumor cell spread and metastasis. In a previous study we observed that suppression of RelA dramatically reduced endogenous uPA synthesis in the human ovarian cancer cell line OV-MZ-6. Because the uPA promoter contains three potential Rel-like protein binding motifs (RRBE, 5'-NF-kappaB, and 3'-NF-kappaB) we conducted the first thorough systematic uPA promoter analysis to examine the direct impact of Rel proteins on uPA gene transcription. Disruption of RRBE resulted in a approximately 40% decrease in uPA promoter activity, mutation of the 5'-NF-kappaB motif led to an additional 20% decrease. The 3'-NF-kappaB motif was not active. Overexpression of RelA significantly enhanced uPA promoter activity, whereas IkappaB-alpha overexpression reduced uPA promoter activity by 40%. These data were supported by the finding that endogenous uPA was also increased sixfold by overexpression of RelA and decreased by 30% upon overexpression of IkappaB-alpha. Transfection of OV-MZ-6 cells with antisense deoxynucleotides directed to RelA expression reduced uPA promoter activity by at least 40%. Our data clearly suggest that by binding to uPA promoter elements, Rel transcripton factors contribute directly to elevated uPA gene expression in human ovarian cancer cells, thereby promoting the multiple functions of uPA during tumor growth and metastasis.

Coagulation/fibrinolysis disorder in patients with severe preeclampsia.

We investigated coagulation/fibrinolysis parameters in 50 patients with severe preeclampsia. The coagulation and clinical indices in patients with severe preeclampsia were 1.595 and 1.414, respectively. These indices show a strong correlation between the clinical status and coagulation/fibrinolysis abnormalities. Among patients with severe preeclampsia, a significant decrease in antithrombin (AT) (p = 0.002) and a significant increase in plasmin inhibitor complex (PIC) (p = 0.002), D-dimer (p < 0.05), and thrombin-antithrombin complex (TAT) (p = 0.001) were observed in cases terminated by cesarean section compared with those cases of vaginal delivery. There was no significant difference in platelet count (Plt), however, deltaPlt (the difference between platelet counts in early gestation and before delivery) was significantly larger (p < 0.05) in cases that ended in cesarean section. Furthermore, among cases terminated by cesarean section, a significant increase in PIC (p < 0.01) was observed when pregnancies were terminated because of maternal factors compared with those terminated because of fetal factors. These results suggest that (1) even in patients with severe preeclampsia, vaginal deliveries can be successful when they display a less hypercoagulable state; (2) a decrease in Plt during pregnancy may be a predictive marker for the termination of pregnancy; and (3) an excessive hypercoagulable state in patients with severe preeclampsia may be associated with the termination of pregnancy, especially when there is an aggravation of maternal factors.

Upregulation of thrombomodulin antigen levels in U937 cells by combined stimulation with estradiol-17beta and vitamin K2 (menaquinone 4).

The expression of thrombomodulin (TM) and tissue factor (TF) antigens by estradiol and vitamin K2 were studied in human leukemic cell lines including U937 (monoblastic leukemia), NB4 (acute promyelocytic leukemia), and HL60 (acute myeloblastic leukemia). Combined stimulation with estradiol-17beta and menaquinone 4 (MK4), homologue of vitamin K2, showed a remarkable increase of total TM antigen level only in U937 cells among these leukemic cell lines, whereas a single treatment of each agent showed a modest or a moderate increase. A synergistic effect of cotreatment with estradiol-17beta and MK4 was observed in an optimum concentration of 1.0 micromol of estradiol-17beta and 1.0 micromol of MK4. Estrogen receptors were detected only in U937 cells among these cell lines, and the competitive assay with an antiestrogenic agent showed a suppression on TM expression in a dose-dependent manner. In the mean time, concerning expression of TF antigens, if at all, only a very slight decrease was observed by costimulation with estradiol-17beta and MK4 in U937 and NB4 cells, whereas all-trans-retinoic acid (ATRA) showed a remarkable decrease in surface TF antigen levels in NB4 cells and also a modest decrease in U937 cells. These findings suggest that estradiol-17beta would up-regulate TM antigen expression via estrogen receptors and in cooperation with MK4, showing a different mechanism from ATRA.

Urokinase induces proliferation of human ovarian cancer cells: characterization of structural elements required for growth factor function.

Ovarian cancer metastasis is associated with an increase in the urokinase-type plasminogen activator (uPA) and its receptor uPAR. We present evidence that binding of uPA to uPAR provokes a mitogenic response in the human ovarian cancer cell line OV-MZ-6 in which endogenous uPA production had been significantly reduced by stable uPA 'antisense' transfection. High molecular weight (HMW) uPA, independent of its enzymatic activity, produced an up to 95% increase in cell number concomitant with 2-fold elevated [3H]thymidine incorporation as did the catalytically inactive but uPAR binding amino-terminal fragment of uPA, ATF. uPA-induced cell proliferation was significantly decreased by blocking uPA/uPAR interaction by the monoclonal antibody IIIF10 and by soluble uPAR. The efficiency of the uPAR binding synthetic peptide cyclo19,31 uPA19-31 to enhance OV-MZ-6 cell growth proved this molecular domain to be the minimal structural determinant for uPA mitogenic activity. Dependence of uPA-provoked cell proliferation on uPAR was further demonstrated in Raji cells which do not express uPAR and were thus not induced by uPA. However, upon transfection with full-length uPAR, Raji cells acquired a significant growth response to HMW uPA and ATF.

The strategy of dural arteriovenous fistula with isolated sinus.

SUMMARY: We treated 7 cases of dural arteriovenous fistulas (dAVF) with isolated sinus by transvenous direct embolization. The fistulas located in the transverse-sigmoid sinus in 5 cases, superior sagittal sinus in 1 case and transverse-sigmoid and superior sagittal sinus in 1 case. The initial symptoms were generalized convulsion in 2 cases, disturbed consciousness in 1 case, tinnitus in 2 cases and transient ischemic attack in 2 cases. We performed sinus packing with coils in all cases following transarterial embolization. All patients improved neurologically after the treatments and AVFs completely disappeared in all cases.

Neonatal alloimmune thrombocytopenia associated with anti-human platelet antigen-3a antibody.

A sister and brother with neonatal alloimmune thrombocytopenic purpura (NAITP) caused by maternal anti-human platelet antigen (HPA)-3a are reported. The children had transient severe thrombocytopenia in the newborn period, and were treated with intravenous gamma-globulin and platelet concentrates from random donors. Although the sister had intracranial hemorrhage on day 2 postnatally, the development of the child has been normal and no neurological sequelae have been observed. The brother only had bloody stool when the platelet count was low, and did not have severe hemorrhagic manifestations. The diagnosis of NAITP was made by the sera from the mother, which contained anti-HPA-3a antibody directed against platelets of the children. The rate of recurrence might be high in this family, because the father of the patients was found to be homozygous for the HPA-3a gene.

Epilepsy in peroxisomal diseases.

PURPOSE: To clarify the electroclinical manifestation of epileptic seizures and the evolution of epilepsy in patients with peroxisomal diseases. METHODS: Retrospective review of the medical records and EEGs of 14 patients with peroxisomal diseases: seven with Zellweger syndrome (ZS), two with neonatal adrenoleukodystrophy (NALD), two with acyl-CoA oxidase deficiency (AOXD), two with bifunctional enzyme deficiency (BFED), and one with rhizomelic chondrodysplasia punctata (RCDP). The diagnoses were made by biochemical analysis and pathological examinations in our laboratory. RESULTS: Patients manifested serious neurologic deficits in the neonatal period or in early or late infancy. Patients with ZS or AOXD had partial motor seizures originating in the arms or legs or corners of the mouth. Their seizures did not culminate in generalized tonic-clonic seizures and were easily controlled by antiepileptic drugs (AEDs). Interictal EEGs of the patients with ZS showed infrequent bilateral independent multifocal spikes, predominantly in the frontal motor cortex and its surrounding regions. The EEGs of patients with AOXD showed interictal fast theta activity, predominantly in the frontocentral regions. Patients with BFED also had partial motor seizures in early infancy, but the seizures were intractable, evolving in one case to myoclonic seizures. Interictal EEGs of patients with BFED showed bilateral independent multifocal spikes that evolved to bilateral diffuse high-voltage slow waves in one case and to a hypsarythmic pattern in another case as the disease progressed. Patients with NALD had intractable tonic seizures or epileptic spasms. Interictal EEGs showed high-voltage slow waves and bilateral independent multifocal spikes, evolving in one patient to a flat pattern. The patient with RCDP, whose interictal EEGs showed frequent multifocal independent spikes, did not have epileptic seizures. CONCLUSIONS: The age of epilepsy onset or the duration of survival is related to the types of seizures occurring in patients with peroxisomal diseases. Neonates or young infants usually have partial motor seizures (facial twitching or clonic convulsions of the arms or legs) of various multifocal origins. Older infants may have generalized seizures at the onset of the disease or evolutionally. Seizure intractability is usually less severe in patients with ZS or AOXD than in patients with NALD or BFED. There is no relation between the electroclinical characteristics of epilepsy and the genetic complementation groups in peroxisomal diseases.

Clinical results and complications of endovascular treatment for intracranial aneurysms.

SUMMARY: Thirty-two patients with intracranial aneurysm were treated via the endovascular approach and clinical results and complications were accessed. The aneurysms were on the anterior circulation, posterior circulation and feeding artery of AVM in 17, 12 and three patients respectively. In 17 (anterior circulation aneurysm), 12 patients underwent parent artery occlusion. There were two complications: cerebral embolism due to premature detachment of balloons and cerebral infarct due to parent artery occlusion. In 12 (posterior circuration aneurysm), four patients had complications: three infarcts due to parent artery occlusion and one aneurysmal bleeding at the interventional procedure. Coil compactions occurred in four cases. There were no complications in cases associated with AVM. Use of detachable balloons for intra-aneurysmal occlusion and intra-aneurysmal embolisation for partially thrombosed giant aneurysm tends to result in ischemic lesions where patient outcome is poor. We must pay attention to avoid these complications even though intra-aneurysmal embolisation using microcoils is currently performed safely.

Vitamin K prophylaxis to prevent neonatal vitamin K deficient intracranial haemorrhage in Shizuoka prefecture.

OBJECTIVE: To compare three methods of vitamin K prophylaxis for neonatal vitamin K deficient intracranial haemorrhage. DESIGN: We designed three strategies for vitamin K prophylaxis: 1. therapeutic administration of vitamin K in a mass screening system using the hepaplastin test; 2. routine oral administration of vitamin K to newborn infants; and 3. administration of vitamin K to lactating mothers during the late neonatal period in addition to the routine method. We evaluated the efficacy of these methods by determining hepaplastin test values at the first month of age. POPULATION: 66,076 full term healthy newborn infants without any complications. RESULTS: Of 55,513 infants in the mass screening system, 3068 infants received vitamin K therapeutically. At the first month of age, in the group where vitamin K was administered therapeutically, 56 infants (1.83%) exhibited low hepaplastin test values (< 40%) despite vitamin K administration. But extremely low values (< 20%), indicating a very high risk of neonatal intracranial haemorrhage, were observed in 34 (0.06%) of 52,445 infants who did not receive vitamin K. In the routine administration system, oral administration of vitamin K twice within the first week of life showed a lower incidence (0.19%) of low level cases than a single administration (1.56%). An additional administration of vitamin K to lactating mothers throughout the late neonatal period showed an effective result.

Case report. Antenatal diagnosis of chorioangioma of the placenta: MR features.

We report a case of chorioangioma of the placenta, in which MR findings were useful in establishing the antenatal diagnosis. Polyhydramnios and a placental tumor that was 5 cm in size were visualized. The tumor had relatively high signal intensities on proton density imaging and T2-weighted imaging and showed partially high intensity signal rims on T1-weighted imaging, especially when using a breath-holding technique. Magnetic resonance imaging has an important role in detection and diagnosis of these lesions, particularly the larger tumors, so that appropriate steps can be taken to deal with the complications that may accompany this tumor.

Inhibition of NF-kappa B-Rel A expression by antisense oligodeoxynucleotides suppresses synthesis of urokinase-type plasminogen activator (uPA) but not its inhibitor PAI-1.

The essential role of urokinase-type plasminogen activator (uPA) in tumor invasion and metastasis stresses the necessity of a fine-tuned cellular control over its expression. It has been shown that changes in uPA directly correlate with changes in cell invasiveness. We examined the role of Rel-related proteins in uPA synthesis by human ovarian cancer cells by inhibiting their expression using the antisense (AS) oligodeoxynucleotide (ODN) technology. Exposure of OV-MZ-6 cells to 10 microM phosphorothioate (PS)-derivatized AS-ODN directed to Rel A led to a maximal 50% decrease of uPA antigen in cell lysates and a 70% reduction in cell cultures supernatants accompanied by a significant transient decline in uPA mRNA levels. Antisense-PS-ODN directed to NF-kappa B1 (p50) or c-rel had no effect on uPA protein expression. AS-PS-ODN directed to Rel A also affected the proteolytic capacity of OV-MZ-6 cells reflected by an approximately 70% decrease in the fibrinolytic capacity of the cells within 24 h compared to untreated controls. AS-PS-ODN directed to I kappa B alpha expression increased uPA in cell culture supernatants up to 50%. uPA receptor (uPAR) production and synthesis of plasminogen activator inhibitor type-1 (PAI-1) were not altered by either AS-PS-ODN applied. Western blot and gel retardation analyses revealed constitutive expression of Rel-related proteins in nuclear protein extracts of OV-MZ-6 cells. Thus these proteins seem to be implicated in uPA regulation and may thereby contribute to tumor spread and metastasis.

Ventricular inversion without transposition of the great arteries: a rare anomaly associated with left-sided (tricuspid) atrioventricular valve atresia and unroofed coronary sinus.

Isolated ventricular inversion with concomitant atresia of the left-sided (tricuspid) atrioventricular valve has previously been reported in the English literature in only two patients, and they died during early infancy. We describe the clinical, two-dimensional echocardiographic and angiocardiographic features in a 10-year-old boy with these rare cardiac anomalies in association with unroofed coronary sinus, which compensated for the restrictive atrial septal defect. This is the first published report of this combination of cardiac anomalies.