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Living With Craniopharyngioma

Last Updated: January 7, 2023

Being diagnosed with a brain tumor can be terrifying. Not knowing what to expect or what life will look like in the coming days is one of the scariest experiences for patients and their families. A craniopharyngioma diagnosis can evoke the same emotions but is perhaps more fearsome due to the extreme rarity of the condition.

It will be critical for the patient to know how to live with the tumor and how treatment could impact their lives and that of their families and caregivers. In this article, we discuss what living with a craniopharyngioma may entail.

Craniopharyngioma — A Quick Overview

Craniopharyngioma is a rare, relatively benign tumor that grows above the pituitary gland. The tumor is thought to grow from the cells of a structure formed during fetal development called the craniopharyngeal duct. Craniopharyngiomas make up 10% to 15% of pituitary tumors and 6% of all brain tumors in children. This type of tumor commonly affects children between the ages of 5 and 14 years and adults over 50 years old. 

The tumor can be solid or cystic. Although they are often benign and do not spread to other parts of the body, they cause serious problems by interfering with the function of the essential surrounding structures. Fortunately, these tumors can be treated by surgery, radiotherapy, or sometimes with chemotherapy. The specific treatment approach varies from patient to patient. 

How Fast Do Craniopharyngiomas Grow?

Craniopharyngiomas are generally slow-growing tumors and take up to 3 years or longer to manifest themselves. Their slow-growing nature contributes to a reported delay of 1 to 2 years between the onset of symptoms and diagnosis

Is Craniopharyngioma Life-Threatening?

Craniopharyngiomas are considered a serious medical condition that may require life-long medical treatment. The tumor develops near your pituitary gland, where it can affect various nerves responsible for vision and structures involved in the formation and release of hormones. Craniopharyngiomas can become severe enough to be life-threatening. 

The tumor itself is not dangerous, but its effects on the neighboring pituitary gland can lead to the underproduction of certain hormones, which can adversely affect development and other vital bodily functions. Some of the medical conditions associated with craniopharyngiomas include: 

  • Growth Deficiency — This condition affects the growth rate in children. In adults, the condition results in low energy, lack of muscle strength, and heart problems. 
  • Gonadotropin Deficiency — Causes delayed puberty in young patients, and women might suffer from amenorrhea (absence of menstrual periods).
  • Adrenocorticotropic hormone deficiency — This deficiency can cause weight loss, loss of appetite, nausea and vomiting, low blood pressure, and muscle weakness. The patient might also feel weak and tired. 
  • Thyroid-stimulating hormone deficiency — Patients might experience fatigue, forgetfulness, and irregular periods in women. 
  • Central diabetes insipidus — People with this condition experience extreme thirst, increased frequency of urination, irregular heartbeat, and dry skin and mucous membranes. 

There are also other medical conditions that can occur if the tumor places pressure on the hypothalamus, like hypothalamic obesity, non-24-hour sleep-wake cycle, and Froehlich's syndrome (delayed puberty, short stature, small testes, obesity). 

Some of these conditions can lead to a shortened lifespan without adequate medical attention and management, which is why craniopharyngiomas can be considered life-threatening. 

How Long Can You Live With Craniopharyngioma?

Following treatment, patients diagnosed with a craniopharyngioma have 10-year survival rates of approximately 90%. This means that 90% of patients live at least 10 years. Many patients live even longer, with some researchers reporting 85% survival rates at 20 years. For better prognosis, medical experts recommend continued medical attention and long-term care because: 

  • The patient may need additional surgery to remove a recurring tumor. 
  • If the tumor or the treatment approach causes problems in the pituitary gland or hypothalamus, hormone-replacement therapy may be necessary to mitigate the hormone deficiencies. 
  • Pituitary and hypothalamic impairments may cause other health problems requiring additional management or lifestyle modifications.

Life After Craniopharyngioma Surgery

Life after surgery begins with the recovery period, which can be several weeks to months depending on the type and success of the surgery. In some cases, patients will need long-term hormone treatment after the surgery. There may neurological deficits caused by the tumor or the surgical treatment, which could adversely affect life after treatment, depending on their severity. Particularly in children, this can lead to impairments in social and school functioning.

Other rare long-term consequences associated with surgical intervention and radiation therapy for craniopharyngiomas can have other neurological implications. Continued monitoring after treatment will be important to ensure that appropriate treatment is initiated if additional conditions develop.

Can a Craniopharyngioma Grow Back?

Craniopharyngiomas can indeed grow back, or recur, even if they appear to be completely removed. Up to 17% of completely removed craniopharyngiomas grow back, and up to 25% to 63% of partially removed tumors grow back. In most cases, the tumors will recur within 2 to 3 years after removal. Following treatment, it is recommended for patients to undergo regular MRI and CT scans to check for any regrowth and allow for early intervention if necessary.

Key Takeaways

Living with a craniopharyngioma can be challenging. Some patients can live a largely normal life, whereas others require long-term medical interventions like hormone therapy or additional treatments due to tumor recurrence. A supportive medical and caregiver team will be essential to managing this condition.

For caregivers, it is crucial to understand the medical needs of a craniopharyngioma patient to best understand how to assist. Some patients can complete easy daily tasks, while others might require more intensive care. Although medical treatments help with managing the physical aspects of this condition, emotional and social impairments will require patience, understanding, and continued support from caregivers and family members.

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