Definition
As it grows, a craniopharyngioma can affect pituitary function, vision, and sometimes hypothalamic function. When large, these tumors can block the flow of cerebrospinal fluid and lead to hydrocephalus (enlargement of the brain’s ventricles).
Craniopharyngiomas may consist of three components:
- Composante solide - partie charnue de la tumeur
- Cystic component - the fluid-filled part of the tumor
- Calcified component
Unlike other tumors, craniopharyngiomas do not always follow a linear course: they may grow in size, remain stable, or even shrink. Watchful waiting may therefore be recommended if the tumor is asymptomatic or causes only mild symptoms.
From a surgical standpoint, these tumors require neurosurgeons who specialize in the sella region and are proficient in all access techniques:
- Endonasal endoscopy
- Transcranial microsurgery
Care is necessarily multidisciplinary, involving neurosurgeons, endocrinologists, ophthalmologists, neuroradiologists, pathologists, and radiation oncologists.
Frequency and age of onset
Craniopharyngioma is a rare tumor, with an estimated incidence of 1 to 2 cases per million people per year. It accounts for 2% of all brain tumors and up to 5 to 10% of brain tumors in children. Both sexes are affected equally.
There are two distinct age groups in which the condition most commonly occurs:
Pediatric formulation
Onset between the ages of 5 and 14. Treated at the pediatric neurosurgery department at Necker Hospital, a referral center for pediatric craniopharyngiomas.Adult form
Onset between the ages of 50 and 70. Treated at the Lariboisière Neurosurgery Department, a center of expertise for craniopharyngiomas in adults.This dual approach underscores the importance of close collaboration between adult and pediatric services, particularly when it comes to coordinating the transition from childhood to adulthood.
Two histological types
Pathological analysis (microscopic examination of tissue samples taken in the operating room) distinguishes between two types of craniopharyngiomas:
Adamantine craniopharyngioma
The most common. A tumor that is often calcified and occurs in both children and adults.Papillary craniopharyngioma
Rarer, exceptionally calcified, occurring only in adults. Associated in 90% of cases with a BRAF p.V600E mutation, making it generally responsive to targeted drug therapy, which leads to a reduction in tumor size in the majority of cases.The underlying cause of craniopharyngiomas is currently unknown.
Common symptoms
- Headaches
- Endocrine dysfunction related to deficient hormone secretion by the pituitary gland
- Visual disturbances
Endocrine signs
Compression of the pituitary gland or pituitary stalk may result in various hormonal deficits:
- General signs — fatigue, depressive syndrome
- Gonadotropic impairment (FSH/LH) — amenorrhea, decreased libido, delayed puberty in children
- Thyrotropic impairment (TSH) — cold sensitivity, constipation, dry skin
- Corticotropic impairment (ACTH) — nausea, vomiting, abdominal pain, arterial hypotension
- Somatotropic impairment (GH) — growth retardation in children
- Lactotropic impairment (Prolactin) — galactorrhea (bilateral milky discharge)
- Posterior pituitary impairment (Desmopressin) — diabetes insipidus: excessive thirst and abundant urination
Visual signs
Compression of the optic nerve or chiasm may result in:
- Blurred vision
- Visual field reduction
Hypothalamic signs
Compression of the hypothalamus may cause:
- Eating behavior disorders — hyperphagia, loss of satiety sensation
- Obesity
- Mood changes, slowing, confusion
- Memory disorders, drowsiness
- Thermoregulation abnormalities
How is the diagnosis made?
In the presence of endocrine, neurological, and/or ophthalmological symptoms, a brain/pituitary MRI is prescribed to confirm the diagnosis.
Endocrine workup (pituitary hormone panel)
A blood test measures pituitary hormones and their targets:
Prolactin—TSHus—T4L—T3L—FSH—LH—Estradiol—Progesterone—Testosterone—ACTH—Cortisol—IGF-1
The objective is to identify hormonal insufficiency related to compression of the pituitary gland or pituitary stalk. An endocrinology consultation completes the management (hormonal replacement therapy if necessary).
Radiological workup
Pituitary MRI is the reference examination — it analyzes the extensions of the craniopharyngioma. A CT scan (angio-CT, bone windows) may complement the evaluation, particularly to identify calcifications.
Ophthalmological workup
Necessary due to the proximity of the visual pathways. It includes visual acuity measurement, visual field examination, and fundoscopy and/or OCT as indicated.
1. Surgical treatment
If the craniopharyngioma is minimally or not symptomatic, initial surveillance may be proposed. However, if it becomes progressive or symptomatic (visual disturbances, hypothalamic disorders), surgical intervention is necessary in a specialized center.
The objective is to remove the maximum amount of tumor while preserving hypothalamic function. When the tumor infiltrates the hypothalamus, a residual portion is generally left in place — this may be treated with radiotherapy if it is progressive or threatens the visual pathways. It is frequently impossible to preserve pituitary function, which requires postoperative hormonal replacement therapy.
Endoscopic endonasal approach
Approach through the nose, preferred in the majority of cases. Minimally invasive technique allowing direct access to the sellar and suprasellar region.Transcranial microsurgery
Superior approach, indicated more rarely depending on tumor configuration. Requires complete mastery of both approaches.This is delicate surgery, but associated with a low complication rate in experienced teams.
2. Hormonal treatment
Management of a craniopharyngioma requires the presence of an endocrinologist at every stage — diagnosis, follow-up, and postoperative care. Hormonal deficits are indeed very common, whether present at diagnosis or occurring after surgery.
Endocrinological management allows for detection and replacement of these deficits with appropriate medication (medication replacing the hormone that the pituitary gland can no longer secrete).
3. Radiotherapy
When the entire tumor cannot be surgically removed, radiotherapy may be indicated to prevent growth of the tumor residue. Radiosurgery may also be proposed for small volumes distant from the optic pathways.
The modalities (proton therapy, conformal radiotherapy, ZapX) are discussed with radiotherapists expert in this pathology:
- Dr. Nataf — Lariboisière
- Dr. Calugaru — Institut Gustave Roussy
- Dr. Jacob — Pitié-Salpêtrière
- Dr. Dhermain — Institut Gustave Roussy
Guide
Your stay is our priority, and we have established a patient pathway program for your surgical schedule, admission and intervention, through to your discharge. Please do not hesitate to contact us if you have any questions.