RESUMO
A giant meningocelic sac has not been usually described in adult patients, due to the fact that it shows a low incidence and few newborn have survived to date though the malformation is benign. We report two cases of patients born with the described malformation and who were not operated at that time, so they reached adulthood with bigger sacs. They needed surgery to remove the sacs, for a different reason. The older one had a fistulous abcess but the LCR did not come out, and it did not improved by the application of topic and antibiotic treatment. The other patient showed a progressive growth of the malformation during the last year, skin hardening and pain. The histological study of the dried sacs proved the existence of a carcinomatous degeneration. In the patients we have treated, it seems that a chronic irritation of the LCR and the appearance of multipotent cells in the meningocele may favour the malignancy of the tissues surrounding the sac. This possible malignancy, already described in the bibliography, suggests a prompt elective surgical treatment of the patients with these congenital lesions as soon as possible.
Assuntos
Carcinoma de Células Escamosas/etiologia , Neoplasias Meníngeas/etiologia , Meningioma/etiologia , Meningocele/complicações , Sarcoma/etiologia , Teratocarcinoma/etiologia , Idoso , Neoplasias Encefálicas/secundário , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/embriologia , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/secundário , Transformação Celular Neoplásica , Cisto Epidérmico/etiologia , Cisto Epidérmico/patologia , Evolução Fatal , Feminino , Humanos , Achados Incidentais , Isquemia/etiologia , Vértebras Lombares/anormalidades , Masculino , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/embriologia , Neoplasias Meníngeas/patologia , Meningioma/diagnóstico , Meningioma/embriologia , Meningioma/patologia , Meningocele/embriologia , Meningocele/patologia , Meningocele/cirurgia , Pessoa de Meia-Idade , Células-Tronco Multipotentes/patologia , Paraplegia/etiologia , Sacro/anormalidades , Sarcoma/diagnóstico , Sarcoma/embriologia , Sarcoma/patologia , Sarcoma/secundário , Medula Espinal/irrigação sanguínea , Disrafismo Espinal/complicações , Teratocarcinoma/diagnóstico , Teratocarcinoma/embriologia , Teratocarcinoma/patologiaRESUMO
INTRODUCTION: The coexistence of non-communicating syringomyelia with extramedullary intradural tumours in surgical series is extremely rare. CASE REPORT: We report the case of a 68-year-old female who had been suffering from predominantly proximal asymmetrical progressive paraparesis, with no involvement of the sphincters; the clinical exploration revealed a band of thermal hypaesthesia D7-D8. Magnetic resonance imaging (MRI), following the administration of gadolinium, confirmed the coexistence of an extramedullary intradural expansive lesion, which had features suggesting it could be a meningioma, and a dorsal syringomyelia. The cavity involved two medullary segments situated above the tumour and there were no associated anomalies at the junction of the medulla and upper spinal cord. Following complete resection of the tumour, which was diagnosed histologically as being a transitional meningioma with abundant psammoma bodies, the patient recovered the ability to walk independently. CONCLUSIONS: MRI with contrast is the best diagnostic means available for the detection and delimitation of the two components in this rare association, although its capacity to detect signs suggesting turbulences in the intracavity fluid, especially in small cavities, has still to be confirmed. The variations in the size and situation of the cavity with respect to the seat of the tumour, and the fact that progress is seldom monitored with MRI in the cases published to date, do not allow us to identify the pathogenic mechanism responsible for this association or to suggest the best type of shunt to prevent a possible expansion of the syringomyelic cavity.