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Pineal Tumors

 

 

 

Signs & Symptoms

The clinical presentation of germ cell tumors depends on:
  • Tumor location and extent
  • Tumor size
  • Patient  age

Raised intracranial pressure is common.

  • Often due to the compression of the Sylvian aqueduct by the tumor.
  • Increased intracranial pressure causes:
    • papilledema
    • headache
    • nausea and vomiting 
  • Can be treated by ventriculoperitoneal shunting (may have risk of dissemination with this) or third ventriculostomy.

Cerebellar signs

  • Due to compression of efferent pathways or the cerebellar peduncles. 
  • Causes ataxia  - Gait abnormalities, unsteadiness and frequent falls.

Eye signs

  • Pineal gland is very close to the pretectum, eye symptoms are common. 
  • The pretectum, which includes the ocular motor centre and pupillary control centre, is between the thalamus and the midbrain.  It is responsible for mediating vertical eye movements. 
  • Problems with the visual pathways in pineal tumors are listed in table below. 
  • Parinaud's Syndrome results in several different visual deficits which include:
    • paralysis of upward gaze
    • reduced pupillary reflexes to light
    • convergence paresis 

Endocrine deficits

  • Associated with germinomas and hypothalamic lesions. 
  • The three main deficits associated with germinoma are:
  • diabetes insipidus
  • visual field defects
  • hypothalamic dysfunction

  • Most other neuroendocrine deficits are secondary deficits. 
  • These may include hypopituitarism, growth failure, and elevated hCG or LH level. 
  • It is far less common to have these secondary neuroendocrine deficits with non-germinoma germ cell tumors. 
  • Choriocarcinoma associated with sexual precocity.  It may also be signaled by an increase in CSF concentrations of hCG and LH.

There are also several non-specific neurological deficits that can appear as a result of a pituitary tumor.

Intracranial hemorrhage is rare but a well-documented side effect of pineal tumors.

  • Occurs in tumors of various histological subtypes as well as in non-neoplastic masses. 
  • The 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-germinoma germ cell tumors tend to have localized clinical features due to pineal mass effect.

Table : Summary of symptoms associated with pineal region tumors.

Increased intracranial pressure

Papilledema

Headaches

Nausea

Vomiting

Cerebellar signs

Gait abnormalities

Frequent falls

Ocular symptoms

Accommodation deficiency

Oculomotor nerve palsy

Parinaud's Syndrome

  • failure of upward gaze
  • convergence paresis
  • reduced pupillary reflexes to light

Failure of downward gaze

Visual field disturbance (mass effect post chiasm)

Lid retraction (midbrain dysfunction)

Pupillary abnormality

Neuroendocrine dysfunction

Precocious puberty

Hypopituitarism

Growth failure

Increased hCG and LH levels

Diabetes insipidus

Non-specific neurologic deficits

Diplopia

Seizures

Choreoathetosis

Ophthalmoplegia

Dementia

Psychosis

 

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