Acessibilidade / Reportar erro

Congenital tumors of the nervous system: II - Craniopharyngiomas

Although craniopharyngiomas are rare intracranial tumors, they constitute the most frequent congenital neoplasms of the nervous system. The authors summarize the development and the further disintegration of the hypophyseal duct, the vestiges of which give rise to the tumor. The classifications of the craniopharyngiomas from the macroscopic (cystic, semi-cystic and solid), histopathological (mucoid epithelial cysts, adamantinomas and squamous cell epitheliomas) and topographic (sellar, supradiaphragmatic and intracraniosellar) standpoints are discussed. The incidence of the growth is higher in the first two decades and in males. The symptomatology is studied according to the site of the tumor, either supradiaphragmatic (hypothalamo-chiasmatic manifestations) or sellar (hypophyseal genital and somatotrophic failure). Radiological changes are very important for the diagnosis, mainly those disclosed by the craniogram (calcifications and sellar erosions), pneumoencephalography, pneumocisternography, air and iodized oil ventriculography, and cerebral angiography. Roentgentherapy of craniopharyngiomas is still a matter of controversy. The surgical management is discussed in detail, the difficulties of the radical excision being emphasized. The high operative mortality rate is ascribed to manipulation of the hypothalamus and to hypophyseal collapse. The association of ACTH and cortisone is advised. Local treatment with radioisotopes seems a promising procedure. The authors report 26 cases of craniopharyngiomas: 14 were supradiaphragmatic and 12 intracraniosellar growths; not one of them was exclusively sellar. Some correlation between age and duration of symptoms is shown. The neurological signs (mainly paralysis of cranial nerves, pyramidal and/or cerebellar syndromes), the ophthalmological manifestations (choked disk, impairment of vision and visual field defects) and the endocrine and hypothalamic disorders (hypopituitarism, diabetes insipidus, obesity) are studied in detail. The results of simple and contrasted radiologic examinations, electroencephalography and cerebrospinal fluid examination are discussed. Twenty-one patients were submitted to surgery (puncture of the cyst and partial excision of the capsule, supplemented in 5 cases by evacuation of the cyst); 32 operations were performed (6 patients had one or more recurrences). In 10 of the most recent cases treatment with ACTH or cortisone was associated. The case mortality rate was 38.5%; there were 5 deaths among 20 patients operated on who could be followed-up (a mortality rate of 25%). The histologic pattern of the tumors was established in 19 cases, including 2 adamantinomas, 11 squamous cell epitheliomas and 6 mixed type craniopharyngiomas. Two other cases displayed malignant features.


Academia Brasileira de Neurologia - ABNEURO R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices Torre Norte, 04101-000 São Paulo SP Brazil, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revista.arquivos@abneuro.org