Ectopic pinealoma in the region of the optic chiasma

The authors report the case of a 31-year-old female patient who 10 years earlier had displayed diabetes insipidus and hyperglycemia and for 4 years galactorrhea and amenorrhea. Since one year her visual acuity had gradually declined to blindness. CT-scan revealed the presence of a tumoural formation in the optic chiasma and hypothalamic region. The histological examination of the operative-removed tumour showed that it was a type B pinealoma. The postoperative course was good for 42 hrs after which the patient died suddenly. Necropsy disclosed the presence of a myocardial infarct. The authors distinguish three categories of ectopic pinealoma: 1. tumours of the pineal gland with an aberrant development at the level of the 3rd ventricle, 2. metastatic pinealoma, and 3. ectopic pinealomas with an independent development in patients with normal pineal gland. The case reported below is of the 3rd category which is also the most veridical.