Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Huber, J; Sovinz, P; Lackner, H; Mokry, M; Eder, H; Urban, C.
Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive.
Klin Padiatr. 2007; 219(2): 91-94.
Doi: 10.1055/s-2007-921559
(- Case Report)
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- Führende Autor*innen der Med Uni Graz
- Höntzsch Julia
- Urban Ernst-Christian
- Co-Autor*innen der Med Uni Graz
- Eder Hans
- Lackner Herwig
- Mokry Michael
- Ritter-Sovinz Petra
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- Abstract:
- BACKGROUND: Diencephalic syndrome (DS) is a rare cause of failure to thrive in early childhood. It is associated with neoplastic lesions of the hypothalamic-optic chiasmatic region. Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy. We describe the clinical course of two children suffering from diencephalic syndrome due to unresectable hypothalamic gliomas and emphasize the importance of chemotherapy as a first-line treatment. PATIENTS AND METHODS: We report about two children, at the age of 21 months and 13 months at diagnosis, who presented with severe dystrophy at 12 months and 6 months respectively. Imaging of the brain showed a suprasellar mass, identified histologically as low grade pilocytic astrocytoma. Both patients were treated with chemotherapy which induced tumor regression and stable disease. RESULTS: The two children gradually gained weight and improved remaining in stable remission. CONCLUSIONS: Diencephalic syndrome caused by a hypothalamic/chiasmatic astrocytoma is a rare cause of failure to thrive in children so that diagnosis is frequently delayed. It should be considered as differential diagnosis in any child with dystrophy despite adequate caloric intake. Since most of these tumors in that specific anatomic site are regarded to be unresectable, chemotherapy including carboplatin and vincristine may reveal clinical improvement in these patients.
- Find related publications in this database (using NLM MeSH Indexing)
- Antineoplastic Combined Chemotherapy Protocols - therapeutic use
- Astrocytoma - diagnosis
- Chemotherapy, Adjuvant - diagnosis
- Child, Preschool - diagnosis
- Combined Modality Therapy - diagnosis
- Diagnosis, Differential - diagnosis
- Failure to Thrive - etiology
- Female - etiology
- Follow-Up Studies - etiology
- Humans - etiology
- Hydrocephalus - etiology
- Hypothalamic Diseases - diagnosis
- Hypothalamic Neoplasms - diagnosis
- Infant - diagnosis
- Neoplasm, Residual - drug therapy
- Optic Chiasm - pathology
- Optic Nerve Neoplasms - diagnosis
- Find related publications in this database (Keywords)
- diencephalic syndrome
- failure to thrive
- hypothalamic/chiasmatic astrocytomas
- chemotherapy