RESUMO
Syringomyelia associated with posterior fossa tumours is a very infrequent combination of pathological entities. The few cases which have been reported generally were asymptomatic in respect of the spinal cavitations. The authors report on a 36-year-old woman with a large extradural posterior fossa epidermoid tumour with a concomitant holocord symptomatic syringomyelia. Some of her symptoms were clearly attributed to the intraspinal cavitation. The lesions were both diagnosed by magnetic resonance imaging (MR). The patient did well after surgery of the brain lesion, with an objective improvement in her neurological status and a complete resolution of the syrinx documented by the MR 7 months after tumour removal. Syringomyelia in this case could be explained by blockage of the cerebrospinal fluid (CSF) circulation at the foramen magnum which in turn resulted in cranio-spinal pressure dissociation. This led to an accumulation of extracellular fluid (ECF) in the central canal, starting cavitation. Consequently, the syrinx was slowly expanded by the long-standing "slosh" effect of the systolic pressure waves. However, also via a distortion mechanism within the posterior fossa a pathologically plugged obex could have contributed to syrinx formation by means of preventing drainage of fluid from the ventricular CSF system.
Assuntos
Neoplasias Infratentoriais/complicações , Neoplasias Infratentoriais/cirurgia , Siringomielia/etiologia , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/etiologia , Pressão do Líquido Cefalorraquidiano , Feminino , Humanos , Imageamento por Ressonância Magnética , Siringomielia/patologia , Resultado do TratamentoRESUMO
Presentamos una serie de 48 pacientes (de 14 a 20 años) con adenoma hipofisario. De éstos, 46 (96 por ciento) presentaban tumores secretantes, 3 enfermedad de Cushing, 9 somatotrofinomas y 34 (29 mujeres y 5 hombres) prolactinomas. Treinta casos fueron diagnosticados como adenomas intraselares (62 por ciento) mientras los restantes 18 (38 por ciento) presentaron expansión extraselar. De los 9 pacientes acromegálicos, 7 desarrollaron bioquímica y clínica típica de la enfermedad mientras 2 fueron exclusivamente diagnosticadas con niveles de GH basales normales, pero pruebas dinámicas anormales. Los prolactinomas fueron no invasivos en mujeres y de crecimiento rápido y de mayor tamaño en hombres. Cuarenta y siete pacientes fueron sometidos a cirugía. Cinco de ellos requirieron craneotomía y el resto fueron abordados por víatranseptoesfenoidal (TSE). Se consiguió remisión de la enfermedad de Cushing, acromegalia y prolactinoma intraselar femeninos. Los resultados en tumores mayores tales como los adenomas no secretantes y prolactinomas masculinos fueron malos luego de haber sido tratados mediante una resección subtotal y los disturbios endocrinológicos persistieron. Nuestros hallazgos demuestran que estos tumores son más agresivos en los jóvenes que en los adultos. Como hubo una estrecha relación entre el tamaño del tumor, su invasividad y el resultado final del paciente, concluímos que el diagnóstico temprano y el tratamiento son esenciales. Las frecuentes consultas de adolescentes tales como menstruaciones irregulares, retraso puberal y alteraciones de crecimiento deberían ser investigadas cuidadosamente y no simplemente consideradas como hechos transitorios o funcionales
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Acromegalia/diagnóstico , Acromegalia/etiologia , Adenoma/complicações , Neoplasias Hipofisárias/classificação , Neoplasias Hipofisárias/diagnóstico , Amenorreia/etiologia , Ergolinas/uso terapêutico , Octreotida/uso terapêutico , Oligomenorreia/etiologia , Prolactinoma/diagnóstico , Prolactinoma/tratamento farmacológico , Prolactina/antagonistas & inibidores , Prolactina/sangue , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/etiologiaRESUMO
We report on a series of 48 patients, ages 14 to 20 year, with hypophyseal adenomas. Of these, 46 (96%) had secreting tumors, 3 had Cushing's disease, 9 had somatotrophinomas, and 34 (29 females and 5 males) had prolactinomas. Thirty cases were diagnosed as intrasellar adenomas (62%) while the remaining eighteen (38%) presented extrasellar expansion. Of 9 acromegalic patients, 7 had typical clinical and biochemical features 2 were exclusively prognatic with normal basal GH levels, but abnormal dynamic tests. Prolactinomas were noninvasive in women and faster growing and more extensive in men. Forty seven patients underwent surgery. Five of these required craniotomy and the rest approached through the sphenoidal bone (TSE). Remission was achieved in Cushing's disease, acromegaly, and female intrasellar prolactinomas. Larger tumors such as nonsecreting adenomas and male prolactinomas showed poor results after undergoing subtotal resections, with persistence of endocrinological disturbances. From our findings it appears that these tumors are aggressive in youth than in adults. Because there was a close relationship between tumor size, invasiveness, and the patients' final outcome, we conclude that early diagnosis and treatment is essential. Frequent complaints in adolescents such as irregular menses, retarded puberty, and growth disorders should be thoroughly investigated and not merely considered as transient or 'functional'.