title: [Russell's syndrome: a case of long-term survival and review of the literature].
abstract: 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. Treatment of choice includes an excisional biopsy with chemotherapy and radiotherapy. We report a clinical case of long-term survival and review of the literature.
authors.affiliation: S. S. Oncologia Pediatrica, Istituto di Clinica Pediatrica, Università di Roma "La Sapienza", Roma, Italia. dr.lory@tiscalinet.it
authors.forename: L
authors.initials: L
authors.lastname: Amoroso
authors.forename: A
authors.initials: A
authors.lastname: Cacchione
authors.forename: D
authors.initials: D
authors.lastname: Valentini
authors.forename: M
authors.initials: M
authors.lastname: Foco
authors.forename: M
authors.initials: M
authors.lastname: Gonfiantini
authors.forename: C
authors.initials: C
authors.lastname: Cappelli
authors.forename: G
authors.initials: G
authors.lastname: Ceccarelli
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001254:Astrocytoma / Q000175:diagnosis* / Q000628:therapy; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D002675:Child, Preschool; D004027:Diencephalon / Q000473:pathology; D006801:Humans; D008297:Male; D013577:Syndrome; D016896:Treatment Outcome