Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic-optic chiasm region

Abstract

related to hypothalamic dysfunction; its main features are emaciation, despite a normal or slightly diminished caloric intake, and an alert appearance. DS has been Leonardo Salviati, M.D. ber 1991 and January 1996, are presented in this article. The children were boys, 2 Division of Diagnostic Imaging, Pediatric Neuroages 6, 7, and 18 months, respectively. Oncology Program of the Department of Pediatrics RESULTS.In all three patients, the initial contrast-enhancing MRIs of the head of the University of Padua, Padua, Italy.showed evidence of tumor dissemination. This finding prompted a study of the 3 Neurobiology Research Unit, Pediatric Neurospine, which in turn showed tumor deposits in all three subjects. Among the 43 Oncology Program of the Department of Pediatpatients younger than 16 years with low grade astroctyoma who consecutively rics of the University of Padua, Padua, Italy.entered the Neuro-Oncology Program during the study period, these 3 patients 4 Division of Pathology, Pediatric Neuro-Oncolwere the only ones who had disseminated tumors. ogy Program of the Department of Pediatrics of CONCLUSIONS. In this study, the hypothesis was formulated that DS and disseminthe University of Padua, Padua, Italy.ated hypothalamic-optic chiasm JPA tend to be more commonly associated than 5 Division of Child Neurology, Pediatric Neuropreviously stated. This study suggests that the initial contrast-enhanced MRI of Oncology Program of the Department of Pediatthe head of a child affected by DS and hypothalamic JPA must be looked at carefully rics of the University of Padua, Padua, Italy.for evidence of tumor dissemination, and that the spine must also be examined cludes old reports of nondiencephalic tumors associated with DS, 1,3 this rare neurologic disease is almost exclusively described in associa-

Pilocytic Astrocytomas in Children: Prognostic Factors—A Retrospective Study of 80 Cases

“…An unfavorable evolution can occur, however, including recurrence after total or partial excision, leptomeningeal spread (1,17,21), and secondary tumors or malignant transformation (5,16), leading to death in 18 to 20% of the cases (10,20). We as well as several other authors have observed that most recurrences occur within 5 years after surgery (2, 9, 10), although late recurrences are possible (3).…”

Pilocytic and Pilomyxoid Hypothalamic/Chiasmatic Astrocytomas

“…CSF dissemination is uncommon for typical PAs, and malignant progression of these lesions is rare (3,(18)(19)(20).…”

Multi focal Pilocytic Astrocytomas With Ependymal Differentiation in the Bilateral Medial Temporal Lobes-Case Report-

“…5,9,14,17) The most common multifocal glial tumors are high-grade astrocytomas, whereas low-grade astrocytomas are less common. 1,5,7,17) In the present case, we believe that the contralateral tumor had the same histology on the basis of the identical radiological characteristics and similar response to the chemotherapy, although no biopsy could be performed.…”

“…Disseminated pilocytic astrocytomas are generally not histologically identical to the original tumor, but were identified based on the radiological findings. 5,10,[14][15][16] In spite of its benign features, pilocytic astrocytoma should be included in the differential diagnosis of multifocal tumors arising in the bilateral temporal lobes, even at initial diagnosis.…”

“…2,3) Multicentric pilocytic astrocytoma occasionally occurs in the same locations. 5,10,[14][15][16] Temporal lobe is a common origin for pilocytic astrocytoma, 3,4,13) but symmetrical, mirrorimaged tumors in the bilateral temporal lobes are unknown.…”