We report the case of a two years old, normally eating child, affected by organic macies and severe dystrophy. After the negative response of blood and laboratory examinations let us exclude a malabsorption syndrome, only the performance of neuro-radiologic exams showed evidence of a subthalamic tumor as the cause of a "Diencephalic syndrome". Diencephalic syndrome or Russell's syndrome is a diencephalic tumor induced disease, which sets in the first time of life. The disease clinical markers are a severe emaciation with appetite preservation and absence or very scarce evidence of any telltale neurological sign. The tumoral histo-pathology most frequently shows a low grade of malignancy astrocytoma, whose eradication is very often difficult because of particular anatomic site. Choice treatment includes an excisional biopsy associated to chemiotheraphy and to radiotherapy. We report a clinical long term survival case and review of literature.
|Translated title of the contribution||Russell's syndrome: A case of long term survival and review of literature|
|Number of pages||4|
|Publication status||Published - Jun 2004|
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