Pineal Germinoma

Nandakumar Srinivasan, MD; Aneesh Pakala, MD; Chandana Mukkamalla, MD; Alok Oswal, MD

Disclosures

South Med J. 2010;103(10):1031-1037. 

In This Article

Discussion

Patients with pineal germinoma present with clinical features of hypothalamic-pituitary axis dysfunction.[4] Parinaud syndrome is an important clinical presentation of pineal germinomas.[12] Parinaud syndrome is caused by direct compression of the quadrigeminal plate, which interrupts the neurons that link the cerebral cortex, the superior calculi, and the occulomotor nuclei. The triad of Parinaud syndrome includes upward gaze palsy, dissociation of pupillary response to light, and accommodation and failure of ocular convergence. Increased intracranial pressure (ICP) due to obstruction to CSF flow by compression of the aqueduct by the pineal germinoma causes obstructive hydrocephalus. The raised ICP can cause headache, projectile vomiting, and papilledema.[13]

Endocrinological manifestations including diabetes insipidus (DI), delayed gonadal function, and precocious puberty are commonly seen in patients with pineal germinomas. The presence of DI is considered to be a marker for suprasellar and third ventricular disease even in the absence of radiographic evidence of suprasellar and ventricular involvement.[6] Neurofibromatosis type 1 and Klinefelter and Down syndrome have been associated with intracranial GCTs.[2,14] There are case reports of pineal germinomas presenting as eating disorders like anorexia nervosa and vomiting, which, in these cases, were erroneously thought to be due to psychogenic causes.[15,16] Pineal germinomas can secrete human chorionic gonadotrophin (HCG) that can stimulate the Leydig cells and increase testosterone production, causing precocious puberty in males.[17] The pineal germinomas can also alter pubertal development by causing the destruction of the normal pineal tissue, which has an inhibitory effect on sexual development. The tumor can also invade the CNS structures that trigger sexual development and some authors suggest that pineal germinomas can directly promote sexual development.[18]

Primary GCTs are associated with an increased risk of skin cancers including melanomas, but this has been attributed to chemotherapy-induced carcinogenesis, since most patients developed melanomas after chemotherapy was initiated.[2] Chang et al reported the involvement of retinal vasculature in the manner consistent with a paraneoplastic syndrome which caused floaters and difficulty in visual tasks that improved with radiation to the primary tumor.[19]

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