Pineal Tumors Clinical Presentation

Updated: Oct 19, 2017
  • Author: Jeffrey N Bruce, MD; Chief Editor: Brian H Kopell, MD  more...
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The clinical syndromes associated with pineal region tumors relate directly to normal pineal anatomy, as well as tumor histology.

Mass lesions in the pineal region that compress adjacent structures result in typical clinical syndromes. One of the most common presentations is headache, nausea, and vomiting caused by aqueductal compression and resultant obstructive hydrocephalus. If left untreated, hydrocephalus may lead progressively to lethargy, obtundation, and death.

Compromise of the superior colliculus, either through direct compression or through tumor invasion, results in a syndrome of vertical gaze palsy that can be associated with pupillary or oculomotor nerve paresis. This eponymic syndrome was first described by the French ophthalmologist Henri Parinaud in the late 1800s and has become virtually pathognomonic for lesions involving the quadrigeminal plate.

Further compression of the periaqueductal gray region may cause mydriasis, convergence spasm, pupillary inequality, and convergence or refractory nystagmus. Impairment of downgaze becomes more pronounced with tumors involving the ventral midbrain. Patients also can present with motor impairment, such as ataxia and dysmetria, resulting from compromise of cerebellar efferent fibers within the superior cerebellar peduncle.

Children with pineal region tumors can present with endocrine malfunction. Hydrocephalus or concurrent suprasellar tumors can cause diabetes insipidus. More specific endocrine syndromes can arise from secretion of hormones by germ cell tumors. Pseudoprecocious puberty caused by beta human chorionic gonadotropin (beta-HCG) can be observed with germ cell tumors in either the pineal or suprasellar region. In a large series of patients with germ cell tumors and suprasellar involvement, 93% of girls older than 12 years had secondary amenorrhea and 33% of patients younger than 15 years had growth arrest.

Pineal apoplexy, bleeding into the tumor area, has been described as a rare presenting feature of pineal region tumors. Hemorrhage into a vascular-rich pineal tumor can occur preoperatively and is a well-described postoperative complication.