Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive.
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.


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.
Huber
J
J
Department of Pediatrics, Division of Pediatric Hematology/Oncology, Medical University Graz, Austria.
Sovinz
P
P
Lackner
H
H
Mokry
M
M
Eder
H
H
Urban
C
C
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001254:Astrocytoma / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005183:Failure to Thrive / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D007027:Hypothalamic Diseases / Q000175:diagnosis* / Q000188:drug therapy; D007029:Hypothalamic Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D007223:Infant; D018365:Neoplasm, Residual / Q000188:drug therapy; D009897:Optic Chiasm / Q000473:pathology; D019574:Optic Nerve Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery