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Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic‐optic chiasm region
Author(s) -
Perilongo Giorgio,
Carollo Carla,
Salviati Leonardo,
Murgia Alessandra,
Pillon Marta,
Basso Giuseppe,
Gardiman Marina,
Laverda AnnaMaria
Publication year - 1997
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/(sici)1097-0142(19970701)80:1<142::aid-cncr19>3.0.co;2-y
Subject(s) - optic chiasm , medicine , optic chiasma , magnetic resonance imaging , pilocytic astrocytoma , pediatrics , radiology , astrocytoma , pathology , glioma , optic nerve , ophthalmology , cancer research
BACKGROUND Diencephalic syndrome (DS) is a complex of signs and symptoms related to hypothalamic dysfunction; its main features are emaciation, despite a normal or slightly diminished caloric intake, and an alert appearance. DS has been almost exclusively described in association with space‐occupying lesions of the hypothalamic‐optic chiasm region, mainly juvenile pilocytic astrocytoma (JPA). A systematic diagnostic approach, including contrast‐enhanced magnetic resonance imaging (MRI) of the child's head, is rapidly expanding our knowledge of this syndrome. METHODS The MRI findings for three children affected by DS associated with biopsy‐proven JPA, consecutively referred to the Pediatric Neuro‐Oncology Program of the Department of Pediatrics at the University of Padua between September 1991 and January 1996, are presented in this article. The children were boys, ages 6, 7, and 18 months, respectively. RESULTS In all three patients, the initial contrast‐enhancing MRIs of the head showed evidence of tumor dissemination. This finding prompted a study of the spine, which in turn showed tumor deposits in all three subjects. Among the 43 patients younger than 16 years with low grade astroctyoma who consecutively entered the Neuro‐Oncology Program during the study period, these 3 patients were the only ones who had disseminated tumors. CONCLUSIONS In this study, the hypothesis was formulated that DS and disseminated hypothalamic‐optic chiasm JPA tend to be more commonly associated than previously stated. This study suggests that the initial contrast‐enhanced MRI of the head of a child affected by DS and hypothalamic JPA must be looked at carefully for evidence of tumor dissemination, and that the spine must also be examined if the findings are positive. Cancer 1997; 80:142‐6. ©; 1997 American Cancer Society.