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All tumours that occur in the pineal gland region are referred to as pineal tumours. They include actual tumours of the pineal gland, such as pineocytoma or pineoblastoma, but also quadrigeminal plate tumours and tumours of the posterior third ventricle. In the pineal region there often occur germ cell tumours. Among the germ cell tumours, germinomas and non-germinoma germ cell tumours are distinguished. The latter are usually highly malignant. Characteristically, certain tumour markers are present in blood and cerebrospinal fluid (alpha-fetoprotein, beta-HCG). Due to the anatomical vicinity of the cerebral ventricles, pineal tumours often cause retention of cerebrospinal fluid with development of hydrocephalus.
Pineal tumours often become evident due to headaches, nausea and vomiting because they lead to occlusive hydrocephalus. During the development of chronic hydrocephalus memory defects and gait disturbances occur. Also visual disturbances and double images can be the first sign of a pineal tumour.
The diagnostics comprises the clinical examination and subsequently an imaging examination of the head. Due to its good resolution of soft tissue, magnetic resonance imaging (MRI) is the method of choice. In order to determine the tumour marker a blood sample and cerebrospinal sample are taken.
Fig. 1: MRI images of pineal tumour (arrows).
A: Ependymoma
B: Glioma of the quadrigeminal plate
C: Pineocytoma
In the case of pineal tumours the surgery is the top priority in the chain of treatment. If it is a benign tumour, e.g. neurocytoma, pineocytoma, or mature teratoma, a cure can be obtained by the complete removal of the tumour. In the case of germinomas and pineoblastomas postoperative radiotherapy always takes place. In the case of non-germinoma germ cell tumours chemotherapy is carried out before surgery; after surgery once again chemotherapy and radiotherapy are applied.
Germinomas are very radiosensitive tumours. A radical tumour removal is not essential. It is important to confirm the diagnosis, which often takes place endoscopically, since one can simultaneously treat the hydrocephalus often caused by the tumour.
Fig. 2. MRI of 22-year-old patient with germinoma in the pineal region (arrows). The patient presented with headaches, nausea and vomiting.
B: The CT showed expansion of the ventricles (hydrocephalus). Due to the hydrocephalus an endoscopic intervention took place with the treatment of hydrocephalus and taking a sample of the tumour.
C: Endoscopic inspection shows the tumour (arrow).
D: Sample taking with seizing forceps.
E: The postoperative CT shows remission of hydrocephalus.
F: The MRI 6 years after radiotherapy shows no recurring tumour. The patient is neurologically non-symptomatic.
Neurocytomas are benign tumours (WHO grade 1) that occur in ventricles. They can lead to occlusive hydrocephalus and induce symptoms of cerebral pressure.
Video 1 (2.1 MB)
Click to start the video.
Fig. 3.
A: MRI of a 49-year-old patient with neurocytoma in the pineal region (arrows). The patient presented with headaches, nausea and vomiting.
B: The MRI shows expansion of the ventricles (hydrocephalus).
C: Video: Due to the hydrocephalus an endoscopic intervention took place with the treatment of hydrocephalus and simultaneous tumour sample taking. For the treatment of hydrocephalus the base of the third ventricle was opened.
D: The postoperative MRI shows the remission of hydrocephalus and the flow of the cerebrospinal fluid through the opening in the base of the third ventricle (arrow).
E: The surgery took place in a sitting position.
F: The video shows the microsurgical tumour removal.
G: The MRI 6 months after surgery shows complete tumour removal. The patient is entirely free of complaints.
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