کرانیوفارنژیوما (Craniopharyngioma) یک تومور مغزی خوش‌خیم (غیر سرطانی) با سرعت رشد پایین است که در نزدیکی غده‌ی هیپوفیز (یک غده‌ی درون ریز کوچک در قاعده‌ی مغز که هورمون‌های مهم متعددی را ترشح می‌کند) و هیپوتالاموس (یک ارگان درون ریز که ترشح هورمون‌ها توسط غده‌ی هیپوفیز را کنترل می‌کند) رشد می‌کند. این تومور اکثراً کودکان بین 5 تا 10 سال سن را درگیر می‌کند با این حال ممکن است در برخی از بزرگسالان نیز دیده شود. با وجود اینکه کرانیوفارنژیوما سرطانی نیست، این غده رشد کرده و ممکن است بر روی بخش‌های همجوار خود در مغز فشار وارد کرده و علائمی مثل تغییرات هورمونی، تغییرات بینایی، رشد آهسته، سردرد، حال تهوع و استفراغ، اختلالات تعادلی و اختلالات شنوایی را ایجاد کند. علت ایجاد این تومور به درستی مشخص نیست با این حال محققین احتمال می‌دهند که این تومور در مراحل اولیه‌ی رشد جنینی در دوران بارداری مادر ایجاد شده و علت ایجاد آن متاپلازی (تغییر شکل غیرطبیعی سلول‌ها) باشد.

آناتومی

غده‌ی هیپوفیز یک ارگان به اندازه‌ی نخود در زیر هیپوتالاموس است که درون استخوان زین ترکی قرار گرفته است. این غده از دو لوب خلفی و قدامی تشکیل شده است. استخوان زین ترکی در فاصله‌ی نزدیکی با ساختارهای ظریف عروقی و عصبی ازجمله کیاسمای بینایی، سینوس غاری و اعصاب سوم، چهارم و ششم مغزی و شاخه‌های افتالمیک و مگزیلاری عصب پنجم قرار دارد. یک توده‌ی بزرگ شونده در غده‌ی هیپوفیز مثل کرانیوفارنژیوما می‌تواند هریک از این ساختارها را تحت تأثیر قرار دهد. ساقه‌ی هیپوفیز غده‌ی هیپوفیز را به هیپوتالاموس متصل می‌کند. و رشته‌های عصبی مهم (اعصاب هیپوفیز – هیپوتالاموس) و عروق خونی کوچکی (عروق باب هیپوتالاموس – هیپوفیز) درون آن قرار گرفته‌اند که می‌توانند هورمون‌ها را وارد لوب‌های خلفی و قدامی هیپوفیز کنند. The compression of the pituitary stalk due to craniopharyngioma affects these nerve fibers and blood vessels and leads to disorders of the endocrine system.

The cause of the disease

The cause of this tumor is not exactly known, however, researchers believe that this tumor was created in the early stages of fetal development during the mother's pregnancy and the cause of its creation is metaplasia (abnormal change in the shape of cells). A craniopharyngioma appears to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (a sac in the primary oral cavity that forms the anterior part of the pituitary gland). Embryonic cells (primary cells) may give rise to craniopharyngioma by abnormal growth of the craniopharyngeal canal or the anterior part of the pituitary gland. These tumors are closely related to another type of cyst called Rathke's fissure cyst, which is sometimes seen in the pituitary gland.

Symptoms

The tumor puts pressure on the pituitary gland (which produces hormones) and the optic nerve with its growth. This problem will send signals from the eyes to the brain. This pressure will cause different symptoms, including:

• Visual changes (loss of vision in one eye or blurred vision)
• Nausea and vomiting
• Balance disorders and difficulty walking
• Headacheswhich will be more severe in the morning
• Drowsiness
• Mood instability and changes Personality
• Slow growth and late puberty
• Weight gain
• Excessive thirst
• Excessive urination
• Difficulty thinking and learning

Diagnosis

An eye doctor will examine your visual field and check your ability to see objects in front of you and objects next to you. In this test, the loss of visual abilities can be a sign that the tumor is putting pressure on the parts of the brain that are responsible for vision. Among the other tests that may be used by the doctor to diagnose the tumor are:

MRI

In this imaging method, powerful magnets and radio waves are used to take pictures of the inside of the brain. First, a substance called gadolinium is injected into one of your veins. This material will lead to a clearer view of the tumor on the image.

CT scan

CT scan is a type of powerful X-ray imaging that provides highly detailed images of the inside of the body. A special dye may be injected into a vein before the imaging to make the tumor more visible in the image.

Blood test

In this test, the levels of hormones and other substances in the blood are measured. Abnormally high levels of hormones can be a sign of tumor putting pressure on the pituitary gland. The only way to be completely sure of having a craniopharyngioma is to do a biopsy. The doctor inserts a needle or other instrument into your skull and removes a small piece of tissue. Then a specialist in the laboratory examines the removed sample under a microscope and detects the presence of tumor cells in it.

Treatment

Different methods of treating this disease are:

Surgery

The main treatment method is surgery to remove as much tumor tissue as possible. This craniopharyngioma surgery can be performed in several ways, which include:

Surgery through the nose

The surgeon makes an incision under the nose or above the gums and inserts his tools into the brain through this way.

Open skull surgery

The surgeon opens part of the skull and removes the tumor. If part of the tumor is in the form of a cyst filled with liquid, the surgeon inserts a tube or catheter into it and empties the cyst. Sometimes surgery may have side effects. These side effects include bleeding and damage to parts of the brain that control hormone secretion, vision, limb movement, and other functions.

Radiotherapy

Radiotherapy is another treatment method for craniopharyngioma. In this method, high-powered X-rays are used to kill cancer cells or prevent their growth. After surgery, the patient may undergo radiation therapy to destroy the remaining tumor cells. These rays enter the body with one of the following two methods:

External radiation therapy

Rays are radiated to the target area using a device outside the body.

Internal radiation therapy

In this method, needles, wires, beads, or other things that emit radiation are placed inside the brain and next to the tumor.

The side effects of radiation therapy include:

• Extreme fatigue
• Redness of the skin
• Nausea
• Hair loss
• Weight gain
• Memory problems

In children, radiation therapy may lead to long-term learning and development disorders. The doctor may wait to use this method, reduce its intensity, or use another type of radiation to reduce the risk. Proton radiation therapy is a new type of this method that uses protons instead of X-rays. This new method can have fewer side effects in children.

Life after treatment

Overview is suitable for people with craniopharyngioma because surgery and other treatment methods usually completely remove the tumor. But this tumor and the treatment methods used for it can affect the level of hormone production in the body. After treatment, you and your baby should see an endocrinologist (a doctor who treats hormone problems). Your child should visit an endocrinologist regularly for many years and be examined. The doctor will help him manage any side effects caused by the tumor or its treatment methods and also diagnose it if the tumor reoccurs. panel-widget-for-3416-0-1">