Ectopic pinealoma in the chiasma region

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Abstract
A classification of 3 types of ectopic pinealomas involving the infundibular region is presented based on the site of origin of the neoplasm and differences in the clinical manifestations of each type. Ectopic pinealomas which involve the infundibular region are usually associated with a characteristic triad of clinical symptoms consisting of diabetes insipidus, visual disturbances and hypo-pituitarism. Diabetes insipidus occurs in almost all cases and is often the initial symptom. Visual disturbances are usually present and include visual field defects of the chiasmal type and optic atrophy. Hypopituitarism is a frequent finding and is associated with dwarfism in tumors arising before puberty and hypogonadism in those occurring after puberty. Pinealomas arising primarily in the infundibular region can be differentiated from those which arise elsewhere and involve the infundibular region by direct extension or metastasis on the basis of absence of symptoms referable to obstruction of the 3d ventricle or symptoms associated with neoplasms in the region of the quadrigeminal plate. Ectopic pinealomas and intracranial teratomas have certain morphologic similarities. The clinical manifestations of these tumors are also quite similar. In the therapy of pinealomas in the chiasmal region, surgical resection of a portion of the tumor is feasible and advantageous in preserving vision and prolonging life. Radiation therapy in conjunction with surgical resection may have a further palliative benefit.

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