Craniopharyngioma Treatment in Delhi, India

Craniopharyngioma is a rare, benign (non-cancerous) brain tumour that typically occurs near the pituitary gland and the hypothalamus. It is most commonly diagnosed in children and adolescents, though it can also occur in adults. Despite being benign, craniopharyngiomas can cause significant health problems due to their location and the pressure they exert on nearby brain structures.

Craniopharyngiomas are classified into two main types:

• Adamantinomatous Craniopharyngioma (ACP): This is the more common type, typically seen in children and adolescents (ages 5-14). ACPs can also occur in adults, but less frequently. These tumours often have a mixed solid and cystic structure, with solid parts containing calcifications.
• Papillary Craniopharyngioma (PCP): This type is less frequent and predominantly affects adults (ages 50-74).PCPs are usually solid tumours with a distinct papillary (finger-like) pattern under microscopic examination.

In rare cases, a mixed or transitional subtype can exist, exhibiting characteristics of both ACP and PCP. This subtype typically behaves more like an adamantinomatous tumour.

The exact cause of craniopharyngiomas remains largely unknown, but they are believed to originate from remnants of Rathke's pouch, an embryonic precursor to the anterior pituitary gland. These remnants, which typically regress during normal development, can sometimes persist and develop into tumours.

Genetic factors play a significant role in the formation of these tumours. For instance, CTNNB1 gene mutations are often found in adamantinomatous craniopharyngiomas, more common in children, while BRAF gene mutations are frequently identified in papillary craniopharyngiomas, more prevalent in adults.

Ongoing research continues to explore the molecular and genetic mechanisms behind craniopharyngiomas, with the goal of improving diagnostic and therapeutic strategies in the future.

Craniopharyngiomas are rare, benign brain tumours that develop near the pituitary gland. Despite being non cancerous, they can cause a variety of symptoms due to their location and potential impact on surrounding structures. Here are the common symptoms associated with craniopharyngiomas:

General Symptoms

• Headaches: Often due to increased intracranial pressure.
• Nausea and Vomiting: Also related to increased intracranial pressure.

Visual Symptoms

• Visual Disturbances: Blurred vision, double vision, or loss of peripheral vision due to pressure on the optic nerves.
• Optic Atrophy: Progressive damage to the optic nerves can lead to vision loss.

Endocrine Symptoms

• Growth Hormone Deficiency: Especially in children, leading to growth failure.
• Hypothyroidism: Low thyroid hormone levels can cause fatigue, weight gain, and other symptoms.
• Adrenal Insufficiency: Can lead to fatigue, muscle weakness, and weight loss.
• Diabetes Insipidus: Characterised by excessive thirst and urination.
• Delayed Puberty or Hypogonadism: In children and adolescents, there may be a delay in sexual development, and in adults, there may be reduced function of the gonads.

Neurological Symptoms

• Cognitive and Behavioral Changes: Memory problems, confusion, or changes in behaviour due to the tumour's pressure on nearby brain structures.
• Hydrocephalus: An accumulation of cerebrospinal fluid in the brain, causing increased pressure and enlargement of the head in infants.

Other Symptoms

• Obesity: Often due to hypothalamic dysfunction.
• Sleep Disturbances: Caused by pressure on the hypothalamus, which regulates sleep.

These symptoms can vary in severity and combination depending on the size and exact location of the tumour, as well as the age of the patient. Early diagnosis and treatment are crucial to manage these symptoms and prevent further complications.

Diagnosing a craniopharyngioma typically involves a multi-step process. Here's a breakdown of the usual course:

Medical History and Physical Exam

Doctors will first discuss the patient's symptoms and medical history in detail. A physical exam will be conducted to assess vision, hearing, balance, coordination, reflexes, and growth and development (in children). This helps identify potential areas of the brain affected by the tumour.

Neurological Exam

This exam specifically focuses on the nervous system by testing reflexes, muscle strength, sensation, coordination, vision, and hearing. It helps pinpoint any neurological abnormalities caused by the tumour's location and size.

Blood Tests

Blood tests can reveal hormonal imbalances that might indicate a pituitary gland dysfunction caused by the tumour. The pituitary gland is responsible for producing several hormones that regulate various bodily functions.

Imaging Tests

Imaging tests are crucial for visualising the brain and the suspected tumour:

• Magnetic Resonance Imaging (MRI): MRI is the preferred imaging modality for diagnosing craniopharyngioma because it provides detailed images of the brain and soft tissues, including the pituitary gland and the tumour.
• Computed Tomography (CT) Scan: A CT scan can also be used to image the brain, but it may not provide the same level of detail as an MRI for visualising soft tissues. However, CT scans might be helpful for surgical planning.

Biopsy (Optional)

In some cases, a biopsy might be recommended to confirm the diagnosis. A small tissue sample is extracted from the tumour, usually during surgery, for microscopic examination by a pathologist. This helps determine the type of craniopharyngioma and guide treatment decisions.

It's important to note that this is a general guideline, and the specific tests performed may vary depending on the individual situation.

Craniopharyngioma treatment typically involves a combination of surgery and radiation therapy. The specific approach depends on various factors like the size, location, and type of the tumour, as well as your overall health. Here's a breakdown of the main treatment options:

Surgery

The primary goal of surgery is to remove as much of the tumour as safely possible. This helps reduce pressure on surrounding structures and improve symptoms.

There are two main surgical approaches for craniopharyngioma:

• Endoscopic endonasal transsphenoidal surgery: This minimally invasive surgery accesses the tumour through the nose and the sphenoid bone at the base of the skull. It's preferred when the tumour is located near the pituitary gland.
• Transcranial surgery: This traditional approach involves opening the skull to access the tumour. It might be necessary for tumours located deeper in the brain.

Complete removal of the tumour may not always be possible, especially if it's entangled with vital structures like nerves or blood vessels. Surgeons will prioritise minimising damage to these structures while removing as much of the tumour as feasible.

Radiation Therapy

Radiation therapy uses high-energy rays to kill cancer cells or shrink the tumour. It might be used:

• After surgery to destroy any remaining tumour cells.
• In cases where surgery is too risky or not feasible.

There are various radiation therapy techniques for craniopharyngioma, including:

• Stereotactic radiosurgery: This delivers a high dose of radiation in a single or few sessions with extreme precision.
• Proton beam therapy: This advanced technique uses protons (charged particles) to target the tumour more precisely, minimising damage to surrounding tissues.
• Conventional external beam radiation therapy: This delivers radiation from a machine outside the body.

Other Treatment Options

• Medications: Medications might be used to manage hormonal imbalances caused by pituitary gland dysfunction due to the tumour.
• Shunt placement: In some cases, a shunt (a thin tube) might be placed to drain excess cerebrospinal fluid (CSF) if the tumour blocks its flow, causing hydrocephalus (accumulation of CSF in the brain).
• Chemotherapy: Chemotherapy is typically not the first-line treatment for craniopharyngioma but may be used in specific situations, such as for aggressive papillary tumours.

The treatment plan for craniopharyngioma is highly individualised, requiring collaboration between neurosurgeons, radiation oncologists, endocrinologists, and other healthcare professionals to optimise outcomes and quality of life for the patient.

Craniopharyngiomas can lead to a range of complications, both from the tumour itself and from its treatment. These complications often arise due to the tumour's proximity to vital brain structures. Here are some common complications:

Neurological Complications

• Vision Loss: Damage to the optic nerves can cause partial or complete vision loss.
• Hydrocephalus: Tumour pressure can obstruct cerebrospinal fluid flow, leading to hydrocephalus, characterised by increased intracranial pressure and swelling of the brain.
• Seizures: Brain irritation or damage can trigger seizures.

Endocrine Complications

• Hormonal Deficiencies: Damage to the pituitary gland can lead to deficiencies in several hormones, causing issues like growth hormone deficiency, hypothyroidism, adrenal insufficiency, and diabetes insipidus.
• Obesity: Hypothalamic damage can disrupt appetite regulation, leading to significant weight gain and obesity.
• Delayed Puberty or Hypogonadism: Children and adolescents may experience delayed puberty, and adults may have reduced sexual function due to hormonal imbalances.

Cognitive and Psychological Complications

• Cognitive Impairment: Tumour effects or treatment can impact memory, attention, and executive function.
• Behavioural Changes: Patients may experience changes in behaviour, including increased irritability, mood swings, or depression.

Surgical Complications

• Infection: Risk of infection at the surgical site or in the brain.
• Bleeding: Potential for bleeding during or after surgery.
• Neurological Damage: Unintended damage to surrounding brain tissue during surgery, leading to various neurological deficits.

Radiation Therapy Complications

• Radiation Necrosis: Damage to healthy brain tissue caused by radiation.
• Secondary Tumours: Long-term risk of developing secondary malignancies due to radiation exposure.

Currently, there are no known preventive measures for craniopharyngiomas, as their exact cause is not well understood. These tumours are thought to arise from embryonic tissue remnants near the pituitary gland, but the factors triggering their development remain unclear. And since they are not linked to lifestyle or environmental factors, typical preventive strategies are not applicable. However, here are a few recommendations for managing risks and early detection.

• Regular Monitoring: For individuals diagnosed with or treated for craniopharyngioma, regular follow-up appointments with imaging studies are crucial to monitor for tumour recurrence or progression.
• Genetic Counselling: While there is no known genetic predisposition for craniopharyngiomas, patients with a family history of similar conditions may benefit from genetic counselling.
• Prompt Medical Attention: Early diagnosis and intervention can help manage symptoms and reduce complications. Patients experiencing symptoms like persistent headaches, vision changes, or hormonal imbalances should seek medical evaluation promptly.

Ongoing research may eventually provide more insights into the causes and potential preventive strategies for craniopharyngiomas, but as of now, early detection and effective management remain the primary focus.

How effective is surgery for treating craniopharyngioma?

Surgery is often the primary treatment for craniopharyngioma and can be highly effective. The goal is to remove as much of the tumour as possible. Complete resection can potentially cure the patient, but this is not always feasible due to the tumour's location near critical brain structures. Subtotal resection, followed by other treatments such as radiation therapy, is a common approach when complete removal is too risky.

Is radiation therapy always necessary after surgery?

Radiation therapy is not always necessary after surgery but is commonly used when complete surgical removal is not possible. It helps target and destroy any remaining tumour cells, reducing the risk of recurrence. In cases where the tumour is partially removed or recurs after initial treatment, radiation therapy becomes an important adjunct to surgery.

Can craniopharyngiomas recur after treatment?

Yes, craniopharyngiomas can recur after treatment, even years after the initial therapy. Regular follow-up with imaging studies is crucial to monitor for recurrence. The likelihood of recurrence depends on factors such as the extent of tumour removal and the effectiveness of any adjunct therapies like radiation.

What hormones might need to be replaced in craniopharyngioma patients?

Patients with craniopharyngiomas often require hormone replacement therapy due to the tumour's impact on the pituitary gland. Hormones that might need to be replaced include:

• Thyroid hormones (for hypothyroidism)
• Cortisol (for adrenal insufficiency)
• Growth hormone
• Sex hormones (testosterone or oestrogen)
• Antidiuretic hormone (for diabetes insipidus)

Can lifestyle changes help manage craniopharyngioma symptoms or treatment side effects?

Yes, lifestyle changes can help manage symptoms and side effects of treatment. These changes might include:

• Healthy diet: Managing weight and supporting overall health.
• Regular exercise: Improving physical function and mood.
• Adequate hydration: Particularly important for those with diabetes insipidus.
• Stress management techniques: Reducing stress can help manage symptoms and improve quality of life.

What are the long-term outcomes for craniopharyngioma patients?

Long-term outcomes for craniopharyngioma patients vary. Many patients lead normal lives, though they may require lifelong hormone replacement therapy and regular medical follow-ups. Cognitive and psychological effects, vision problems, and other complications can impact quality of life, but supportive care and rehabilitation can improve outcomes.

Can craniopharyngioma affect fertility?

Yes, craniopharyngiomas can affect fertility, particularly if the tumour or its treatment damages the pituitary gland or hypothalamus, leading to hormonal imbalances that affect reproductive function. Hormone replacement therapy can help manage these effects, and fertility treatments may be an option for those wishing to conceive.

What advancements are being made in the treatment of craniopharyngiomas?

Advancements in craniopharyngioma treatment include:

• Minimally invasive surgical techniques: Improving safety and reducing recovery time.
• Targeted therapies: Research is ongoing into drugs that specifically target tumour cells.
• Advanced radiation techniques: Such as proton therapy and stereotactic radiosurgery, which minimise damage to surrounding healthy tissue.
• Biological therapies: Investigating the use of biological agents to target tumour growth at the molecular level.

How does craniopharyngioma impact cognitive function and learning in children?

Craniopharyngioma can impact cognitive function and learning in children due to its effects on brain structures involved in memory, attention, and executive function. Treatment and the tumour itself can lead to difficulties with learning, memory, and behaviour. Early intervention with educational support and neuropsychological rehabilitation can help manage these impacts.

How does craniopharyngioma affect quality of life?

Craniopharyngioma can significantly affect quality of life through various symptoms and treatment side effects, including hormonal imbalances, vision loss, cognitive and emotional changes, and physical discomfort. Ongoing medical care, psychological support, and rehabilitation are essential to improve and maintain quality of life for patients.

What support resources are available for craniopharyngioma patients and their families?

Support resources for craniopharyngioma patients and their families include:

• Patient advocacy groups: Providing information, support, and advocacy.
• Counselling services: Offering emotional and psychological support.
• Support groups: Connecting patients and families with others who have similar experiences.
• Educational resources: Providing information about the disease and its management.

How can craniopharyngioma affect emotional and mental health?

Craniopharyngioma can affect emotional and mental health through its physical symptoms, hormonal imbalances, and the stress of managing a chronic condition. Patients may experience anxiety, depression, mood swings, and cognitive difficulties. Psychological support, counselling, and appropriate medical management are crucial for addressing these issues.

What is the role of genetic counselling for families with a history of craniopharyngioma?

While craniopharyngiomas are not typically associated with genetic inheritance, genetic counselling can provide valuable information for families concerned about the risk of brain tumours. It can help assess familial risk, provide support, and guide decisions about monitoring and preventive measures.

How important is multidisciplinary care in managing craniopharyngioma?

Multidisciplinary care is essential in managing craniopharyngioma due to the complex nature of the disease and its treatment. A team approach involving neurosurgeons, endocrinologists, radiation oncologists, ophthalmologists, neuropsychologists, and other specialists ensures comprehensive care that addresses all aspects of the patient's health and well-being.

Are there specific challenges in treating craniopharyngiomas in paediatric patients compared to adults?

Yes, treating craniopharyngiomas in paediatric patients presents specific challenges, including:

• Growth and development concerns: Ensuring normal growth and development while managing the tumour.
• Hormonal impact: Addressing hormonal imbalances that affect growth, puberty, and overall health.
• Long-term follow-up: Children require long-term monitoring and care to manage potential late effects of treatment.
• Psychosocial support: Providing support for emotional and psychological development.

What follow-up care is required for craniopharyngioma patients after treatment?

Follow-up care for craniopharyngioma patients includes:

• Regular imaging studies: To monitor for tumour recurrence.
• Hormone level assessments: To adjust hormone replacement therapy as needed.
• Vision exams: To detect and manage any changes in vision.
• Cognitive and psychological assessments: To address any ongoing or new issues.
• Rehabilitation services: To support physical, cognitive, and emotional recovery.

Reviewed By Dr. Husain Bhati Consultant- Brain & Spine Surgery on 22 July 2024.