Chemotherapeutic treatment of the diencephalic syndrome. A case report

Tumors of the hypothalamus present with a variety of clinical syndromes. Such syndromes appear to be both age and histology dependent. The diencephalic syndrome, an entity classically seen in infancy, presents as failure to thrive. In essentially all cases the pathologic substrate is a tumor with a predominance of gliomas. The traditional approach to treatment of the diencephalic syndrome has been surgery both for decompression and pathologic diagnosis followed by irradiation. Because of the tumor location, surgery is often confined to biopsy rather than radical extirpation. Furthermore, in infancy focal brain irradiation is not without significant morbidity. Within this context, we would like to present a case discussion regarding a 27-month-old boy with a hypothalamic-midbrain protoplasmic glioma treated with primary chemotherapy after surgical biopsy and pathologic documentation. The schedule of drugs utilized was based on the hypothesis of biochemical modulation of nitrosourea chemotherapy. Successful response to therapy was documented by sequential computed tomography (CT) studies and serial neurologic examinations beginning at age 5 months and every 2 months thereafter. The response of a single patient with the diencephalic syndrome treated primarily with chemotherapy makes extrapolation premature. Rather, the authors suggest chemotherapy as an alternative to focal brain irradiation. Such therapy could be utilized either in the event of recurrent tumor or as late consolidation after primary treatment with chemotherapy. The latter approach would allow a patient to have radiation therapy deferred until a later age at which time morbidity attending brain irradiation may be minimized.