Pineoblastoma

What is Pineoblastoma?

The condition known as pineoblastoma is a malignancy of the pineal gland. This type of malignancy make up about 1% of all intracranial tumors in adults and about 3-8% of all diagnosed brain tumors in children [1, 2].

The pineal gland is an endocrine organ that is found near the center of the brain. It is around 1cm in size and reddish-gray in color. The main function of this gland is to produce melatonin that affects the circadian rhythm and regulate the reproductive hormones of the body. Pineoblastoma is considered to be the most-aggressive tumor among all pineal gland tumors and comprise around 24-50% of these cases.

They originate from neuroectodermal cells and is found to be the least differentiated malignancy of the pineal gland. Also, pineoblastoma has a higher tendency to involve the brain structures that are adjacent to the pineal gland. The 5-year survival rate for patients with this malignancy is between 60-65% [1, 3, 4, 5]. Figure 2 shows a pineoblastoma in a brain.

Figure 2- Pineoblastoma

Cause and Risk Factors

The cause of pineoblastoma is still unknown but its development is associated with abnormalities in the chromosomes. More research is required to identify which errors that directly cause this malignancy [2].

Risk factors for this condition include inheritance of the following genetic conditions: turcot syndrome, fanconi anemia, Li-Fraumeni syndrome, Rubinstein-Taybi syndrome and Nevoid basal cell carcinoma syndrome [6].
Signs and Symptoms

Some of the initial symptoms that will present are associated with the increase in the intracranial pressure. The patient may experience nausea, headache and vomiting. Pineoblastoma may also compress the cerebral aqueduct and lead to the development of obstructive hydrocephalus. If this is not managed, the hydrocephalus may lead to lethargy and death. Other symptoms which may present in the patient are hormonal problems, double vision and difficulty in looking up [1, 4].
Diagnosis

Health history and physical examination

The patient may be brought to the physician due to the presenting symptoms. The physician will have to take note when the symptoms have started developing and whether these have intensified over the time period. The history of patient’s previous health conditions will be elicited by the physician as well. The physical examination will identify other symptoms the patient may experience [1, 6].

Imaging tests

Performing a MRI scan to the patient is important in establishing the diagnosis of pineoblastoma. This test will be able to reveal the location and the size of the tumor have developed. The MRI can also assist in the assessment of the amount of hydrocephalus [1, 6, 7].

Biopsy

A biopsy may also be performed to confirm the diagnosis and rule out other tumors that may develop in the same area. Minimally invasive techniques are utilized by neurosurgeons to obtain tissue samples for analysis [1, 6, 7].

Chemical analysis

Pineoblastoma release certain chemical such as AFP, CEA and Beta-HCG into the CSF. Laboratory tests may be able to detect the level of these chemicals in the CSF and it may remove the need for a biopsy to be performed [6].

Treatment

Surgery

Surgery is the treatment of choice to remove the pineoblastoma and it is usually done using minimally invasive techniques. These procedures are very difficult but there are specialists who are experts in these type of surgery. The excision will also assist in the definitive diagnosis of pineoblastoma. The hydrocephalus can also be managed through either a ventriculostomy or placing of a shunt [1, 2, 6, 7].

Radiation therapy

The treatment for pineoblastoma includes radiation therapy but it is not done to patients who are less than 3 years of age. This is because radiation have a long-term effect in the patient’s cognitive development. Even if low doses of radiation are given to these patients, there are severe potential complications such as endocrine dysfunction, secondary tumorigenesis and cerebral necrosis [1, 2, 6, 7].

Chemotherapy

This mode of therapy uses anticancer drugs to achieve its intended effect. The combination of chemotherapeutic drugs depend on the tumor to be removed. Chemotherapy may also be performed in combination with surgery and radiation therapy to ensure that all tumor cells are removed from the body [1, 2, 6, 7].
References

• Bruce, J. (2015, October 27). Pineal Tumors. Retrieved from Medscape: http://emedicine.medscape.com/article/249945-overview#a7
  American Brain Tumor Association. (2014). Pineal Tumors. Retrieved from American Brain Tumor Association: http://www.abta.org/brain-tumor-information/types-of-tumors/pineal.html?referrer=https://www.google.co.nz/
• Sargis, R. M. (2014, June 10). An Overview of the Pineal Gland. Retrieved from Endocrine Web: http://www.endocrineweb.com/endocrinology/overview-pineal-gland
  Di Muzio, B., & Gaillard, F. (2016). Pineoblastoma. Retrieved from Radiopaedia: http://radiopaedia.org/articles/pineoblastoma
• St. Jude Research Hospital. (2016). Pineoblastoma. Retrieved from St. Jude Research Hospital: https://www.stjude.org/disease/pineoblastoma.html
• National Cancer Institute. (2016, January 28). Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)–Patient Version. Retrieved from National Cancer Institute: http://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq
• UCLA Health. (2016). Pineoblastoma. Retrieved from UCLA Health: http://neurosurgery.ucla.edu/pineoblastoma

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