Craniopharyngioma (Pediatric)

Craniopharyngiomas are slow-growing (grade I) tumors that form above the pituitary gland but below the brain. They can press on the pituitary gland and hypothalamus, leading to hormone problems, and because they sit near the optic nerves, they can affect vision. Their tendency to attach to these structures makes complete removal challenging. These are rare tumors, occurring in about 0.5 to 2 cases per million each year.

Types & Stages

• Adamantinomatous Variant (ACP). The most common type, making up 85 to 95 percent of cases. ACP occurs most often in children and adolescents, though it can appear in adults.
   
• Papillary Variant (PCP). Less common, accounting for five to 15 percent of cases. PCP typically occurs in adults.

Signs & Symptoms

The most common symptoms include:

• Persistent or recurring headaches
• Vision problems
• Hormone (endocrine) problems or deficiencies

Diagnosis

Tests used to diagnose a craniopharyngioma include:

• Physical and neurological exam. Your doctor reviews your child's symptoms, health and family history, then checks neurological function such as reflexes, coordination, strength and sensation.
   
• Magnetic Resonance Imaging (MRI)
   
• Computed Tomography (CT) scan
   
• Blood tests. Hormone testing checks for deficiencies such as thyroid, growth hormone and adrenal insufficiency caused by the tumor's proximity to the pituitary gland.
   
• Visual field testing. Identifies vision deficits caused by pressure or damage to the optic nerves.

Treatment

Treatment options include:

Surgery

For most patients, surgery is the first treatment because it removes as much of the tumor as possible and relieves pressure on the brain. Two common approaches are:

• Transsphenoidal surgery. The neurosurgeon reaches the tumor through the nasal passages and sphenoid sinus, accessing the pituitary gland from beneath the brain while avoiding key brain structures.
   
• Craniotomy. The surgeon makes a scalp incision, removes a portion of the skull and accesses the brain, often using microscopes, endoscopes or intraoperative imaging to remove the tumor safely.

Nonsurgical Treatments

When the entire tumor cannot be removed, radiation therapy can destroy remaining tumor cells and stop further growth. Stereotactic radiosurgery is a non-invasive option for small tumors or those in hard-to-reach areas. It delivers a focused radiation beam to the tumor while sparing nearby healthy tissue. Common types include:

• Gamma Knife radiosurgery. Focuses radiation beams on a precise area, affecting only nearby cells. It treats lesions in the head and uses a metal frame attached to the skull for accurate targeting.
   
• CyberKnife radiosurgery. Uses image-guided robotics to deliver precise, targeted energy beams that destroy tumor tissue while sparing healthy tissue.

Causes & Risk Factors

Most craniopharyngiomas occur sporadically, meaning the cause is unknown. They are believed to develop from tissue left over in the embryo during formation of the craniopharyngeal duct.

• Age. These tumors are more common in children but can appear in adults. They peak in childhood between ages 5 and 14, and again in adults ages 50 to 74.

Screening

There is no routine screening for craniopharyngiomas. Diagnosis relies on recognizing symptoms early and seeking prompt medical evaluation.