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Craniopharyngioma

  • A craniopharyngioma is a tumor of the brain that commonly affects children.

    • The tumor grows in the area of the pituitary gland and the optic nerves, and frequently grows up into the base of your child's brain.
    • It grows from cells that in the developing embryo had helped to form the normal pituitary gland.
    • Portions of the tumor frequently have calcium deposits.
    • The fluid portions of the tumors can reach very large size, and occasionally extend into both sides of the brain.

    Emerging treatments for cysts

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center researchers are constantly developing new and better treatment options. If cysts formed by the tumor are extremely large and complex, we may utilize certain chemotherapeutic agents, such as Bleomycin, to slow or stop cyst growth by injecting them directly into the cyst themselves. There is relatively limited experience with this technique in North America, but we have used it in a few patients with varying success.

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center

    44 Binney Street, 3rd Floor
    Boston MA 02115 

    888-PEDI-ONC 

  • What are the symptoms of a craniopharyngioma?

    These tumors cause symptoms in three major ways.

    Impaired hormonal functions

    The pituitary gland controls nearly all the hormones in your child's body. Since this tumor grows near the gland, it can affect production of vital hormones.

    • The most common manifestation of this hormone effect is a fall-off in normal growth rate due to a lack of growth hormone.

    Increased brain pressure

    These tumors can grow up into the base of the brain, obstructing the chambers in the brain through which the fluid in the brain circulates.

    • This obstruction results in headache, nausea, and vomiting-all symptoms of hydrocephalus, a build-up of fluid inside the brain.

    Loss of vision

    It's possible for these tumors to put gradually increasing pressure on the optic nerves, which leads to severe vision loss in one or both eyes.

    • Many children will be initially diagnosed when they fail a vision test at school or when it is suddenly noted by their parents that their vision is dramatically reduced.
  • How is a craniopharyngioma diagnosed?

    When some children with large craniopharyngiomas are initially diagnosed, they may be quite ill with increased pressure in their brain and there really is not much time to do any special additional diagnostic testing.

    • In this case, surgery must be carried out rapidly to reduce the brain pressure.

    If time is available, however, there are some tests that are helpful to the treating physicians.

    CT scan of the brain - This lets your child's doctor determine the extent of the calcium build-up within the tumor. Calcium deposits, which are not seen well on MRI, often denote areas of the tumor that may be difficult to remove.

    • Knowing the location of these areas can help the surgeon plan the operation more effectively.

    Endocrinologic and ophthalmologic evaluations - Evaluating your child's body function in these areas can help her doctors design an optimal post-surgery management plan.

  • How craniopharyngioma treated?

    Almost always, we attempt to remove as much of the tumor as is safely possible and, hopefully, cure your child by removing all of it. We have been able to do this in about 65 percent of our patients.

    In the vast majority of children with craniopharyngiomas, the tumors grow from the pituitary stalk region; meaning that the removal of the tumor invariably results in a full and complete pituitary hormonal deficit. These children, therefore, require life-long replacement with hormones and must remain under the care of an endocrinologist throughout their growth and development.

    Even if surgery is completely successful in removing the tumor, there is still a possibility that the tumor could regrow from small fragments of the tumor inadvertently left behind at the operation. So your child may require another operation to remove these recurrences.

    Surgery may not sound pleasant, but the risks of surgery are usually well worth the benefits of reducing the intracranial pressure, preserving vision and achieving a long-term cure.

    Why surgery instead of radiation?

    Radiation therapy is an important part of treatment strategy in many cases. Although these tumors are "benign," their growth can be frequently slowed or stopped by radiation therapy.

    However, many children who come to the hospital with a craniopharyngioma are often very sick with pressure symptoms or visual deficits. The radiation therapy does not work quickly enough to reduce these symptoms and surgery is often required immediately to deal with the pressure of the tumor and cyst.

    Radiation treatment is an issue that you should explore in detail with your child's physicians when treatment options are being reviewed. Your child's age, symptoms, preoperative hormonal status, and size and configuration of the tumor are among the many factors which need to be taken into consideration when tumor treatment is planned.

    My surgeon has recommended focused radiation treatment (stereotactic radiosurgery, Gamma Knife, proton beam) for my child's tumor; is this reasonable?

    For some children, these highly focused, single-dose radiation techniques are a useful method to treat areas of tumor that cannot be successfully removed by any other means.

    Because these techniques are intended to destroy all tissue that has been targeted, they're best used only for small tumor areas that are not touching structures whose function would be harmed by this type of radiation.

    For example, if the tumor is near or touching the brain stem or optic nerves, these treatments may not be safe. It's definitely something to talk about in detail with your child's physician.

    What is the recommended long-term care for my child?

    Our experience is that your child will need lifetime follow-up care because the tumor can regrow. For this reason, we will follow your child yearly until 10 years after surgery and then every two years indefinitely.

    Children should visit a survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of cancer
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the Stop and Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, children are able to meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

    • Our pediatric brain tumor survivorship clinic is held weekly.
    • In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists or alternative/complementary therapy specialists.
    • Alternative/complementary therapy specialists, and school liaison and psychosocial personnel from the pediatric brain tumor team are also available.
    • Children needing rehabilitation may meet with speech, physical, and occupational therapists during and after treatments.
  • Dear Dr. Mandell,

    Our son, Jonathan, was diagnosed with a craniopharyngioma brain tumor and had his first surgery in 2008 at an institution in Maryland where we reside. The tumor unfortunately recurred within three months. While doing our research, the name Michael Scott, MD, Children’s neurosurgeon-in-chief, came up frequently. We contacted him for advice and he was kind enough to meet with us within a few days. He was gentle, kind, patient, thorough and efficient, and our decision to proceed with a second surgery with him was made before we left his office. In March, Jonathan underwent a very long, difficult surgery with Dr. Scott and Edward Smith, MD. Jonathan’s recovery went very smoothly.

    Looking back, we realized that everything at Children’s was in place and prepared from the moment we arrived, so our experience was unmatched to any other health care institution. The pre-op staff, anesthesiologists, PICU team, 9th floor team, physical therapists and spiritual advisors were all excellent and we felt were attentive to all our needs and concerns. Our deepest and most sincere praises and gratitude go out to Dr. Scott and Dr. Smith. We could not have asked for better pairs of hands to work on our son. There’s no doubt in our minds that we had the best from a technical standpoint, but also from a humanitarian standpoint.

    We really want to convey that ever since Jonathan was diagnosed with his tumor, we’ve been living with anxiety, fear and a sense of despair. However, since our experience at Children’s, we no longer feel the same way. Thank you to everyone for making a difficult time bearable for us.

    Sincerely,

    Bobby Altman and Aklil Getachew

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