Aggressive fibromatosis involving the cranial base in children.

OBJECTIVE: Aggressive fibromatoses are proliferations of fibrous tissue that infiltrate surrounding tissues and tend to recur after surgical resection. We report a series of five children with aggressive fibromatosis involving the cranial base. METHODS: A search of the database maintained by the Medical Informatics Department at The University of Texas M.D. Anderson Cancer Center identified 78 patients with aggressive fibromatosis of the head and neck. Of these patients, five demonstrated involvement of the cranial base. All five were children. A retrospective review of the charts, diagnostic images, and pathological findings for these five patients was performed. RESULTS: Surgery, radiotherapy, chemotherapy, and hormonal therapy were the treatment methods used. Four patients underwent gross total resections, but tumor cells were present at the margins in all cases. Two of these four patients experienced recurrences; one patient was cured of disease by a second surgical resection and radiotherapy, and the other underwent a second gross total resection. The fifth patient underwent a subtotal resection. He has experienced four recurrences and continues to live with residual disease. One transformation to a Grade 2 fibrosarcoma was observed. CONCLUSION: Fibromatoses of the cranial base are rare and seem to be most common in the pediatric age group. Gross total resection, preferably in the first attempt, should be the goal. Radiotherapy and hormonal therapy are useful adjunctive methods for the treatment of aggressive fibromatosis. Fibromatoses that involve the cranial base are most likely to require a multidisciplinary treatment approach.

Treatment of dissecting pseudoaneurysm of the cervical internal carotid artery using a wall stent and detachable coils: case report.

OBJECTIVE AND IMPORTANCE: This case illustrates the use of an endovascular stent and coiling combination to treat a giant wide-necked pseudoaneurysm of the cervical internal carotid artery. CLINICAL PRESENTATION: A 20-year-old male patient presented with a bilateral dissection of the cervical internal carotid artery and a right giant wide-necked pseudoaneurysm of the cervical segment of the internal carotid artery after a high-speed motor vehicle collision. INTERVENTION: After failing conservative therapy, the patient was treated by endovascular placement of a Palmaz wall stent at the level of the pseudoaneurysm and filling of the pseudoaneurysm with multiple Guglielmi detachable coils. Obliteration of the pseudoaneurysm was achieved, and patency of the right internal carotid artery was maintained. CONCLUSION: The patient's neurological symptoms resolved completely after treatment, and he sustained no neurological complications during the 20-month follow-up period. This case illustrates the successful treatment of a wide-based giant pseudoaneurysm by using a combination of an endovascular stent and coil embolization.

Adenovirus-mediated gene therapy of experimental gliomas.

The efficacy of adenovirus (ADV)-mediated gene therapy to treat brain tumors was tested in a syngeneic glioma model. Tumor cells were transduced in situ with a replication-defective ADV carrying the herpes simplex virus thymidine kinase (HSV-tk) gene controlled by the Rous sarcoma virus promoter. Expression of the HSV-tk gene enables the transduced cell to convert the drug ganciclovir to a form that is cytotoxic to dividing cells. Tumors were generated in Fischer 344 rats by stereotaxic implantation of 9L gliosarcoma cells into the caudate nucleus. Eight days later, the tumors were injected either with the ADV carrying the HSV-tk (ADV-tk) gene or a control ADV vector containing the beta-galactosidase (ADV-beta gal) gene and the rats were treated with either ganciclovir or saline. Tumor size was measured 20 days after implantation of 9L cells or at death. Rats treated with ADV-beta gal and ganciclovir or with ADV-tk and saline had large tumors. No tumors were detected in animals treated with ADV-tk and with ganciclovir at doses > or = 80 mg/kg. An infiltrate of macrophages and lymphocytes at the injection site in animals treated with ADV-tk and ganciclovir indicated an active local immune reaction. In survival studies, all animals treated with ADV-tk and ganciclovir have remained alive longer than 80 and up to 120 days after tumor induction whereas all untreated animals died by 22 days. These results demonstrate that ADV-mediated transfer of HSV-tk to glioma cells in vivo confers sensitivity to ganciclovir, and represents a potential method of treatment of brain tumors.

Pylephlebitis associated with diverticulitis.

We have reported the cases of two patients who had acute pylephlebitis associated with portal vein thrombosis and septic hepatic emboli as a result of right colonic diverticulitis. Although rare, pylephlebitis is a treatable but often lethal complication of intra-abdominal sepsis. Several bacterial pathogens, especially Escherichia coli are associated with pylephlebitis. Early suspicion and prompt antibiotic therapy can lead to resolution of portal vein thrombosis and hepatic abscess formation, resulting in full recovery for the patient. Surgery may not be required. Our two patients received ampicillin--the best first-line drug--until specific antibiotic therapy could be given. Early administration of a broad spectrum antibiotic is essential.

CT-guided stereotactic biopsy of nonenhancing brain lesions.

The majority of computer tomography stereotactic biopsy lesions enhance after administration of intravenous contrast, whereas patients with nonenhancing lesions are often followed conservatively or undergo craniotomies. There are few studies showing the effectiveness of stereotactic biopsies of nonenhancing cerebral lesions. Stereotactic biopsies were performed on 19 patients with lesions that did not enhance on CT after intravenous contrast. Pathological diagnoses were made in 90% (17/19) of patients. Four HIV-positive patients had progressive multifocal leukoencephalopathy, 11 patients had gliomas (4 astrocytomas, 6 anaplastic astrocytomas, and 1 ganglioma), 1 had multiple sclerosis, and 1 had herpes encephalitis. In 2 patients multiple biopsies revealed only gliosis. There was no morbidity or mortality. Stereotactic biopsies for nonenhancing brain lesions have a high diagnostic yield and can favorably alter the treatment course.

Pathologic changes in olfactory neurons in Alzheimer's disease.

Olfactory deficits and degenerative changes in central olfactory pathways are prominent in patients with Alzheimer's disease (AD). We hypothesized that peripheral olfactory neurons that reside in the nasal epithelium would show degenerative changes similar to the characteristic pathologic features of AD brain. Immunohistochemical studies of nasal tissue taken at autopsy reveal extensive degeneration in the sensory epithelium as well as abnormal neurites that share immunoreactive epitopes with dystrophic neurites and neurofibrillary tangles of the AD brain. The neuritic masses are stained with well-characterized monoclonal antibodies that do not normally stain olfactory neurons but which are very reactive with dystrophic neuritic structures and neurofibrillary tangles in AD brain. These include antibodies to phosphorylated and nonphosphorylated neurofilament subunits, tau, and also ALZ50, which is characteristically reactive with AD but not with normal brains. Such changes are present in 81% of AD patients. Similar accumulations of ectopic neurites are found in the olfactory epithelium of about 22% of non-demented patients. Preliminary statistical analysis fails to reveal any age-linked association. It has been proposed that the aged monkey is a good model for AD inasmuch as amyloid accumulations similar to those of humans are found in monkey brain. We examined a series of 13 rhesus monkeys, including aged animals with behavioral deficits. Although the olfactory epithelium was very similar to that of humans, no abnormal olfactory structures were observed. Aged rhesus monkeys do not appear to be a good model for the neuritic abnormalities of AD.

Lower airway smooth muscle contraction induced by Ptychodiscus brevis (red tide) toxin.

Ptychodiscus brevis toxin (PBTX) is produced by the organism Ptychodiscus brevis. This toxin causes a phenomenon that has come to be known as Florida red tide. It also stimulates neuronal sodium channels, resulting in activation of the cholinergic and adrenergic nerve endings of the autonomic nervous system in upper airway smooth muscle and rat vas deferens, respectively, as previously reported. It is the cholinergic stimulating action that has been implicated as a possible "triggering" event in bronchial asthma. This article concerns the investigation of whether PBTX may also affect lower airways and by what mechanism any contractile response to PBTX in lower airways may be induced. PBTX was found to elicit contractions in isolated canine lower airway smooth muscle. The threshold concentration was 0.15 microgram/ml, the peak response occurred at 6.0 micrograms/ml, and the concentration causing half-maximal response of the group was 0.57 microgram/ml. Pharmacologic analysis demonstrated that atropine (10(-6) mol/L) blocked the response to both PBTX and acetylcholine, tetrodotoxin (10(-7) mol/L) blocked the response to PBTX but not to acetylcholine, and verapamil (10(-5) mol/L) blocked the response to PBTX and reduced the response to acetylcholine. Four consecutive contractile responses to PBTX (3 micrograms/ml) produced rapid tachyphylaxis. The fourth contraction was 60% less than the initial response. A fifth response to PBTX after exposure to indomethacin (2.8 X 10(-6) mol/L) was increased and resulted in a contraction that was only 25% less than the initial response. The exogenous addition of prostaglandins (PG), PGE1 and PGE2, to indomethacin-treated lower airway strips selectively suppressed the contractile response to PBTX. Other PGs tested (PGA2, PGB2, PGD2, PGF2 alpha and PGI2) did not affect the PBTX response. These results indicate that PBTX produces spasm in lower airway smooth muscle and that it does this by stimulation of sodium channels in the cholinergic nerve fibers. The results also demonstrate a rapid reduction in the contractile response to PBTX. The results also demonstrate that the reduction is mediated by PGs of the E series.

[Mapping of the cardiac electric activity. Applications to experimental surgery]. / Cartographie de l'activité électrique cardiaque. Applications à la chirurgie expérimentale.

The results reported by French and other groups of workers have shown that peroperative epicardial mapping is a valuable technique for locating the accessory pathway in the Wolff-Parkinson-White syndrome and also in the surgical treatment of certain forms of chronic ventricular tachycardia. The aim of this study was to develop a completely automatic method of mapping potentials recorded at many points during the same systole. A system of acquisition and automatic treatment of 256 cardiac potentials called SATAPEC was constructed at the University of Caen. The system comprises specialized recorders, a multiplexing system on 256 leads with analogical-digital conversion and a PDP 11-23 mini computer. The results presented are based on the location of the His bundle and the right bundle branch in the dog from recordings of the H potential. The recording device with 240 (20 x 12) measuring points is a silk net on which 50 micrometers diameter silver wires are attached. The wires are insulated except at their tips and are separated one from another by a distance of 2 mm. After infundibulotomy the recording device was placed over the interventricular or right interatrial septum. The 240 unipolar electrograms were recorded during a single systole. The programmes which have been developed automatically produce maps of the amplitude of the H potential. The computer detects the presence or absence of a H potential on each electrogram and then displays on a cathode ray tube points of variable size indicating the locations of the electrodes which have recorded a H potential.(ABSTRACT TRUNCATED AT 250 WORDS)