RESUMEN
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.
Asunto(s)
Fibromatosis Agresiva/cirugía , Neoplasias de la Base del Cráneo/cirugía , Adolescente , Niño , Preescolar , Terapia Combinada , Femenino , Fibromatosis Agresiva/diagnóstico , Fibromatosis Agresiva/patología , Fibromatosis Agresiva/radioterapia , Humanos , Lactante , Masculino , Grupo de Atención al Paciente , Reoperación , Estudios Retrospectivos , Base del Cráneo/patología , Base del Cráneo/cirugía , Neoplasias de la Base del Cráneo/diagnóstico , Neoplasias de la Base del Cráneo/patología , Neoplasias de la Base del Cráneo/radioterapia , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Aneurisma Falso/terapia , Disección Aórtica/terapia , Traumatismos de las Arterias Carótidas , Embolización Terapéutica/instrumentación , Stents , Adulto , Disección Aórtica/diagnóstico por imagen , Aneurisma Falso/diagnóstico por imagen , Angiografía de Substracción Digital , Arteria Carótida Interna/diagnóstico por imagen , Angiografía Cerebral , Humanos , Masculino , Examen NeurológicoRESUMEN
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.
Asunto(s)
Adenoviridae , Neoplasias Encefálicas/terapia , Ganciclovir/uso terapéutico , Terapia Genética/métodos , Glioma/terapia , Animales , Virus del Sarcoma Aviar/enzimología , Neoplasias Encefálicas/patología , Relación Dosis-Respuesta a Droga , Femenino , Glioma/patología , Macrófagos/patología , Regiones Promotoras Genéticas , Ratas , Ratas Endogámicas F344 , Secuencias Repetitivas de Ácidos Nucleicos , Simplexvirus/genética , Timidina Quinasa/genética , Trasplante IsogénicoRESUMEN
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.
Asunto(s)
Infecciones Bacterianas/etiología , Diverticulitis/complicaciones , Enfermedades Intestinales/complicaciones , Flebitis/etiología , Vena Porta , Enfermedad Aguda , Enfermedades del Ciego/complicaciones , Diverticulitis del Colon/complicaciones , Femenino , Humanos , Masculino , Persona de Mediana Edad , Flebitis/diagnóstico , Flebitis/microbiología , Trombosis/complicacionesRESUMEN
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.