title: Disseminated pilocytic astrocytoma involving brain stem and diencephalon: a history of atypical eating disorder and diagnostic delay.
abstract: The association of weight loss and pediatric brain tumors that affect the diencephalon or brain stem with weight loss is a recognized, but not fully understood phenomenon. Tumors located in the hypothalamic region may induce the diencephalic syndrome (DS), which is characterized by profound emaciation with almost complete loss of subcutaneous fatty tissue. Tumors that compress or infiltrate the brain stem rarely cause both psychological disturbance and emaciation. The clinical presentation may be different, depending on the location of the lesion and age of the patient. In this report we present an unusual case of severe emaciation in a 4(9)/(12)-year-old girl with a juvenile pilocytic astrocytoma of the hypothalamic region and brain stem with neuroaxis dissemination. This case illustrates the importance of considering intracranial mass-lesions in the differential diagnosis of weight loss, psychological disturbance and atypical eating disorder. We discuss the importance of tumor multifocality and the role of patient age in the clinical presentation with reference to the literature.
authors.affiliation: Department of General Pediatrics, Heinrich-Heine-University, Düsseldorf, Germany.
authors.forename: Felix
authors.initials: F
authors.lastname: Distelmaier
authors.forename: Gisela
authors.initials: G
authors.lastname: Janssen
authors.forename: Ertan
authors.initials: E
authors.lastname: Mayatepek
authors.forename: Jörg
authors.initials: J
authors.lastname: Schaper
authors.forename: Ulrich
authors.initials: U
authors.lastname: Göbel
authors.forename: Thorsten
authors.initials: T
authors.lastname: Rosenbaum
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001254:Astrocytoma / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D001932:Brain Neoplasms / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004614:Emaciation / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D001068:Feeding and Eating Disorders / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D019959:Feeding and Eating Disorders of Childhood / Q000175:diagnosis; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000473:pathology; D013120:Spinal Cord Neoplasms / Q000150:complications / Q000556:secondary; D016896:Treatment Outcome