Pineal gland tumours

Only 1 in every 100 brain tumours (1%) are pineal tumours.

The pineal gland is found at the back of the third ventricle of the brain. Ventricles are fluid-filled spaces within the brain.

Pineal-region tumours can be made up of different types of cells. The most common type of tumour in the pineal region is known as a germinoma or germ cell tumour.  Germinomas develop from germ cells (cells in a very early stage of development). They are fast-growing and may often spread to other parts of the brain.

Other types of pineal tumour include: astrocytomas, teratomas, meningiomas, pineocytomas and pineoblastomas.

Although this type of tumour is more common in adults, it can occur in children. For unknown reasons, it is more common in men than in women.

Signs & Symptoms

The clinical presentation of germ cell tumors depends on:

  • Tumor location and extent
  • Tumor size
  • Patient  age

 

Signs and Symptoms in Pineal tumors:

Problem

Signs and symptoms

Raised Intracranial Pressure

Common

 

Often due to the compression of the Sylvian aqueduct by the tumor

 

Increased intracranial pressure causes:

  • Headache (worse in morning)
  • Nausea and vomiting
  • On examination papilledema

 

Can be treated by:

  • Third ventriculostomy
  • Ventriculoperitoneal shunting (may have risk of dissemination with this)

Visual problems

Pineal gland is very close to the pretectum – eye symptoms are common 

 

The pretectum:

  • Includes the ocular motor centre and pupillary control centre
  • Between the thalamus and the midbrain
  • Responsible for mediating vertical eye movements

 

Problems with the visual pathways in pineal tumors:

  • Accommodation deficiency
  • Oculomotor nerve palsy
  • Failure of downward gaze
  • Visual field disturbance (mass effect post chiasm)
  • Lid retraction (midbrain dysfunction)
  • Pupillary abnormality

 

Classic problem with Pineal tumor:

  • Parinaud’s Syndrome results in several different visual deficits which include:
    • paralysis of upward gaze
    • reduced pupillary reflexes to light
    • convergence paresis 

Cerebellar signs

  • Gait abnormalities, unsteadiness and frequent falls
  • Due to pressure on cerebellar peduncles

Endocrine deficits

Associated with germinomas and hypothalamic lesions 

 

Common deficits associated with germinoma:

 

Other secondary neuroendocrine deficits: 

  • Include hypopituitarism, growth failure, and elevated hCG or LH level.
  • Precocious puberty
  • Far less common to have secondary neuroendocrine deficits with non-germinoma germ cell tumors. 
  • Choriocarcinoma can be associated with sexual precocity (also may have an increase in CSF concentrations of hCG and LH).

Intracranial hemorrhage

Rare but a well-documented side effect of pineal tumors:

  • Occurs in tumors of various histological subtypes as well as in non-neoplastic masses. 
  • Bleeding may cause pineal apoplexy or subarachnoid hemorrhage. 
  • Massive hemorrhage into choriocarcinomas and endodermal sinus tumors is most frequent. 
  • Tumors of the pineal parenchyma are also very vascular. 
  • Repeated subarachnoid hemorrhage is associated with germinomas and pineocytomas. 

Non-specific neurologic deficits

  • Diplopia
  • Seizures
  • Choreoathetosis
  • Ophthalmoplegia
  • Dementia
  • Psychosis

         

Non-germinoma germ cell tumors tend to have localized clinical features due to pineal mass effect.

pineal tumour

http://aiimsnets.org/NeurosurgeryEducation/NeurosurgicalSpecialties/Neuro-oncology/Surgical%20Approaches%20to%20Pineal%20Region%20Tumors%202012/Surgical%20Approaches%20to%20Pineal%20Region%20Tumors%202012.pdf