Craniopharyngioma

Introduction to craniopharyngioma Craniopharyngioma is a common embryonic residual tissue tumor developed from the epithelial cells of the craniopharyngioma formed by the ectoderm. It is the most common congenital tumor in the brain and the second in the sellar tumor. Occurs in children, adults are less common, occur in the saddle, accounting for 5 to 7% of the entire intracranial tumor, accounting for 12 to 13% of children with intracranial tumors, the main clinical features of the hypothalamic-pituitary dysfunction Increased intracranial pressure, visual and visual impairment, diabetes insipidus and neurological and psychiatric symptoms can be clearly diagnosed by CT scan. The main treatment is surgical removal of the tumor. basic knowledge The proportion of illness: 0.021% Susceptible people: good for children Mode of infection: non-infectious Complications: meningitis, disturbance of consciousness, hydrocephalus, diabetes insipidus, shock, epilepsy, upper gastrointestinal bleeding

Cause of craniopharyngioma

Genetic factors (30%)

The disease is a congenital disease, which grows slowly. When the normal embryo develops, the elongated tube connecting the Rathke sac and the original oral cavity is the craniopharyngioma. This tube gradually disappears with the development of the embryo, and the residual part of the anterior wall of the Rathke sac In the nodules, the residual squamous epithelial cells of the degenerated craniopharyngioma may become the origin of the occurrence of craniopharyngioma, so craniopharyngioma can occur in the pharynx, sphenoid sinus, saddle, saddle and third ventricle, Some can invade the posterior fossa.

Physical and chemical factors (30%)

Of the many physical mutagenic factors, the most widely used and effective is the ray. The radiation used for mutagenesis includes ionizing radiation and non-ionizing radiation. Some chemicals, like radiation, can cause genetic mutations in organisms.

Biological factors (30%)

The activity of any other animal, plant or microbe that affects the growth, morphology, development and distribution of the organism is a type of ecological factor that can be divided into intraspecific and interspecific relationships.

Pathogenesis

1. Pathogenesis Regarding the organization of craniopharyngioma, there are currently two theories that are generally accepted.

(1) Congenital Residual Theory: This is a widely accepted theory of histogenesis. Erdheim first observed that there are residual squamous epithelial cells in the nodules of the normal pituitary. It is believed that craniopharyngioma originates from these residual epithelial cells. In the second week of the embryonic period, the original oral cavity topped up to form a deep blind pocket called the Rathke bag. As it further developed, the lower part of the Rathke bag became narrow and thin, which is called the craniopharyngioma. Or pituitary tube, under normal circumstances, the embryo gradually disappeared in 7-8 weeks after the embryo, and there are often small nests of epithelial cells in the development process, which becomes the tissue source of craniopharyngioma.

(2) Scaly epithelial metaplasia: In 1955, Luse and Kernohan observed 1364 autopsy pituitary glands, and found that only 24% had squamous epithelial cell nests, and their incidence increased with age, and those under 20 years old The incidence of squamous cell nests is very low. Therefore, they believe that the squamous cell nest is a product of pituitary cell metaplasia, not embryonic residues. In addition, humans have observed a mixture of pituitary gland cells and squamous epithelial cells, and see There is an excess between the two, and this discovery also supports the metaphysical doctrine.

2. Pathological changes The volume of craniopharyngioma is generally large, the shape of the tumor is often spherical, irregular, or nodular expansion, no obvious envelope, clear boundaries, significant differences in range, mostly cystic multi-atrial Or partial cystic, a few are substantial, only a small number of small cysts, tumor grayish red, cyst fluid can be yellow, brown, brown or colorless, such as cyst rupture, cyst fluid overflow, can cause meningitis and arachnoid Inflammation, cystic people are mostly located on the saddle, and the cystic part is often above the parenchyma. The surface of the cyst wall is smooth and the thickness is different. The thin one can be as translucent, with multiple grayish white or yellowish brown calcification spots or calcified spots. It can be ossified into an eggshell-like shape. The capsule content is degenerated liquefied epithelial cell debris (keratin-like substance). The cyst fluid is oily or golden yellow liquid containing flickering floating cholesterol crystals, generally 10-30 ml. More than 100ml, the tumor parenchyma is often located in the lower posterior, nodular, containing calcification, sometimes dense and hard, often associated with intracranial important blood vessels, pituitary stalk, visual pathway and anterior third ventricle Tightening and pressing the above structure, swollen Tumor can also cause the glial reaction zone of brain tissue to form a pseudo-envelope, sometimes it can be papillary into the hypothalamus. When the tumor is pulled by surgery, it may cause damage to the hypothalamus. The substantial tumor is located in the saddle or the third ventricle. It is smaller than the cystic person.

Tumor tissue morphology can be divided into two types: enamel type and scaly type. The enamel type is more common, mainly in children. The outermost layer of this type is columnar epithelial cells, which gradually move toward the center and the outer layer is fence-like. The stratified cells are arranged in loose stellate cells. The tumor tissue often has degenerative changes, keratinization and small cysts. After the detached cells in the sac absorb calcium, many scattered calcifications are prominent features of the craniopharyngioma. Almost all craniopharyngioma Calcification can be seen under the microscope. In most cases, calcification can be found during radiology. The craniopharyngioma often protrudes from the papillary into adjacent brain tissue (especially the hypothalamus), making the tumor closely connected with these brain tissues. Therefore, the operation is often not easy to completely remove. The scaly papilla type is composed of well-differentiated squamous epithelial cells, which are rich in fibrovascular matrix, and the cell membrane is naturally cleaved or a pseudo-papillary shape is formed due to the cracking of the lesion. Non-enamel type keratinized beads, calcification, inflammatory reaction and cholesterol deposition, this type is mostly solid tumors, occasionally reported craniopharyngioma grows rapidly, invasive recurrence, but Mathematics were not considered to be malignant transformation, some of the electron microscope anaplastic tumor manifestations, in tissue culture despite the tendency to balloon, but almost no mitotic activity.

There are differences in the location of blood supply to craniopharyngioma. The blood supply to the tumor on the saddle is mainly from the small artery of the anterior circulation of the Willis ring. It is also thought that there is blood supply directly from the internal carotid artery and the posterior communicating artery, but the craniopharynx The tube tumor does not receive blood supply from the posterior cerebral artery (or basilar artery) unless the tumor is close to the bottom of the third ventricle where the blood vessel supplies blood. The blood supply to the tumor inside the saddle is from the small penetrating artery of the internal carotid artery of the cavernous sinus.

The tumor grows around and can oppress the optic nerve, the pituitary gland, the bottom of the third ventricle, the hypothalamus, and even block the interventricular pores on one or both sides to cause obstructive hydrocephalus. Most of the saddle-type tumors are substantial and volume. Small, early limited to the saddle can directly compress the pituitary, and later growth can affect the optic nerve, the optic chiasm and the third ventricle.

Craniopharyngioma prevention

1. Psychological nursing craniopharyngioma occurs mostly in children and young people. Their psychological tolerance is poor. Once they are diagnosed, the psychological burden is very heavy, which is easy to produce fear and pessimism. In addition, craniotomy has certain risks, and patients often have Feeling uneasy, afraid and irritated, affecting rest and sleep, and even refusing surgery, the nurse must patiently answer the patient's various questions, relieve the patient's ideological concerns, and introduce successful cases to establish the confidence of patients to overcome the disease.

2. Assessment of visual field of vision for craniopharyngioma due to direct oppression of the optic nerve, optic chiasm and optic tract, 70 to 80% of patients have visual acuity, visual field disorder, nurses can initially understand the patient's vision, visual field, specific methods : Let the patient look straight ahead, use the fingers to move in the upper, lower, left and right directions, check the patient's visual field, and use the hand index at different distances in front of the patient (eg 1 m, 2 m, 3 m, etc.). Visual acuity was assessed and compared with postoperative visual acuity after recording.

3. Observation of hypothalamic lesions The development of craniopharyngioma to the saddle is enlarged to the bottom of the third ventricle, and the hypothalamus is compressed. The result may be symptoms of diabetes insipidus, high fever, coma, etc. The patient's urine volume 3 days before surgery provides a digital basis for postoperative observation of diabetes insipidus.

4. For patients with high intracranial pressure, dehydrating agents and diuretics should be given immediately to reduce intracranial pressure. Such patients should be prepared as soon as possible for surgery.

5. Before surgery, patients with hypopituitarism should pay attention to replenish a sufficient amount of glucocorticoids to avoid pituitary crisis. Other pituitary hormones may not be replenished, because many patients can recover from postoperative pituitary function. If there is still hypopituitarism after surgery, appropriate treatment should be given.

Craniopharyngeal complications Complications meningitis conscious disorder hydrocephalus diabetes insipidus shock epilepsy upper gastrointestinal bleeding

Craniopharyngioma has a slow growth and a long course of disease, which mainly damages the structure of the lower and surrounding hypothalamus, causing endocrine dysfunction, visual acuity, visual field damage and increased intracranial pressure. The treatment is mainly surgery. Common postoperative complications are as follows:

Central high fever

The patient's high fever persists, is in a coma state, and the prognosis is poor. It is usually treated symptomatically. The reasons may be: 1 hypothalamic function damage during craniopharyngioma resection, causing hyperthermia due to thermoregulatory dysfunction; 2 cystic tumor The cyst fluid stimulates the meninges and hypothalamus to produce aseptic meningitis; 3 the blood cerebrospinal fluid stimulation caused by surgery causes fever.

Closely observe the heat type and duration after surgery, distinguish between central hyperthermia and lungs, high fever caused by urinary tract infection, and use hypnotic drugs with caution in patients with fever to prevent disturbance of consciousness, post-head pillow ice pack, ice cap or body ice The blanket, continuous anal temperature monitoring, body temperature is quickly controlled below 38.5 °C, which is caused by hypothalamic injury during surgery.

2. Disorder of consciousness

Mainly due to impaired hypothalamic or increased intracranial pressure, increased intracranial pressure: 1 postoperative clot obstruction of the aqueduct caused by hydrocephalus; 2 surgical hemostasis caused by subdural hematoma or epidural hematoma; 3 surgical stimulation Or electrolyte imbalance caused secondary cerebral edema, nurses should closely observe changes in the patient's mind and pupils, especially within 72 hours after surgery to observe whether the patient has nausea, vomiting and increased wound tension, neck stiffness and other symptoms, keep the drainage tube unobstructed, pay attention to Observe the color and quantity of drainage fluid. For those with conscious disturbance, use Glasgow coma scoring method to evaluate the degree of consciousness, timely discover and timely and correct treatment.

3. Diabetes insipidus

This complication is almost inevitable in patients with total tumor resection or radical subtotal resection, which is caused by injury to the pituitary stalk during surgery. After the pituitary stalk is damaged, the release of ADH is three-phase. Initially, the pituitary stalk is affected. Depletion of ADH after injury reduces the collapse of the urine; after the degeneration of the axonal extremity of the pituitary gland releases super-physiological ADH, this release process is usually 48-96 h after pituitary stalk injury, if the patient is given long-acting (oil agent) resistance Diuretic preparation (usually given short-acting vasopressin) may cause endogenous release of ADH and cause a decrease in renal function; when the hormone released by the degenerated nerve endings is depleted, it will reoccurate, generally diabetes insipidus It can be recovered from several days to 2 weeks, but there are also a few cases of permanent diabetes insipidus. The treatment is as follows:

(1) Focus on observing patients with polydipsia, polyuria, polydipsia and other urine output, urine specific gravity, record 24h in and out, according to the amount of fluid added to the liquid, urine volume <5000ml / d, can be used without drugs, conscious people Drink more water; demented, 2 to 3 hours after surgery to give indwelling stomach tube, add water and nutrition, urine volume > 5000ml / d, urine specific gravity <1.005, subcutaneous injection of pituitrin 5U subcutaneous, once / d, or urine Disruption 0.3ml, 1 time / d, intramuscular injection, urine collapse light usually first given hydrochlorothiazide (hydrochlorothiazide), carbamazepine oral treatment, severe cases can be applied short-acting vasopressin, during which attention should be paid to control the amount of fluid To prevent water poisoning (at this time the patient may have edema, convulsions, etc.).

(2) Regularly measure the serum sodium, potassium, chlorine, carbon dioxide binding rate, and pH and blood urea nitrogen. The electrolyte is measured every 12 hours after 3 to 5 days after surgery. If the electrolyte is lost, it can be supplemented normally; Retention (increased blood sodium and increased osmotic pressure) should limit sodium intake; patients with low sodium and low chlorine should be supplemented with sodium chloride to prevent brain edema; to prevent hypokalemia from oral potassium chloride, 1000ml of chlorination Potassium 1g, in addition, must maintain potassium, calcium, sugar at normal levels.

Circulatory failure

Preoperative patients with obvious pituitary dysfunction, prone to acute adrenal cortical failure after surgery, the patient is in a state of shock, the treatment is to add hormones before surgery, postoperative failure to give high doses of adrenal cortex hormones, which can not only Reducing the crisis can also reduce hypothalamic response and cerebral edema. It also has a positive effect on the prevention of central hyperthermia. However, in order to reduce complications such as infection and gastrointestinal bleeding, the dosage should be gradually reduced 4 days after surgery. The maintenance dose was gradually stopped after 2 weeks (except for those with obvious pituitary dysfunction).

5. Epilepsy

Due to surgical trauma and hypothalamic traction, epilepsy occurred after anesthesia was awake, oral administration of phenytoin sodium 0.1g, 3 times / d before surgery; intramuscular injection of 10mg or phenobarbital 0.1g for prevention, surgery After monitoring the EEG or observing the patient's oral twitching, eyelid tremor, finger twitching and other signs, the abnormality is detected immediately before the convulsion, repeated medication during seizures, while keeping the airway open, giving oxygen inhalation to prevent brain tissue hypoxia .

6. Gastrointestinal bleeding

The patient may have melena, hematemesis, or even acute gastric perforation after reflex caused by gastric mucosal erosion after ulceration of the lower thalamus, ulceration caused by upper gastrointestinal bleeding and a large number of corticosteroids.

Postoperative application of cimetidine, strict observation of blood pressure, pulse and stool color, indwelling gastric tube, observe the digestion of food in the stomach and the color of gastric juice, sudden hematemesis, melena, pulse rate, blood transfusion, ice brine wash Stomach, stomach injection of 1000IU thrombin, once / 4h, and the application of omeprazole, cimetidine, etc., blood transfusion, application of hemostatic agents, H2 receptor blockers, and fasting, gastrointestinal decompression , stop hormones, etc., if necessary, surgical treatment, so that bleeding is controlled in a timely manner.

7. Aseptic meningitis

The contents of the tumor sac are caused by the overflow of the meninges during the operation. For this reason, the tumor should be removed as much as possible during the operation, and the cyst can be repeatedly washed with saline. After the operation, the cerebrospinal fluid can be discharged through the lumbar puncture. Symptoms such as fever can also help.

8. Visual impairment

Intraoperative injury to the visual pathway and the blood vessels it supplies can cause visual impairment, especially in the anterior-posterior type of tumor, which should be noted.

9. Pituitary function is low

In particular, there are pituitary dysfunction before surgery, generally difficult to recover, children with growth retardation, short stature, sexual dysplasia, etc., treatment of thyroid hormones and other drugs and strengthen exercise, is expected to have some degree of recovery, but not grasp Big.

10. Other

The complications of intracavitary radionuclide internal irradiation in craniopharyngioma can be summarized as: damage optic nerve crossing, optic tract, hypothalamus, radiation brain tissue necrosis, vascular embolization, and radiotherapy-induced tumors, etc. Tumor recurrence or death.

Symptoms of craniopharyngioma Common symptoms: fatigue, weight loss, polydipsia, refusal to eat, polyuria, inattention, high fever, intracranial hypertension, nipple atrophy, ... severe headache

Craniopharyngioma can be seen at any age, but it is most common in 6 to 14 years old. Most craniopharyngioma are intermittently growing, so the overall growth of the tumor is slow, and the symptoms develop slowly. A small number of craniopharyngioma grows. Rapid, its disease progress is also faster, its clinical manifestations include the following aspects: tumor occupying effect and high intracranial pressure caused by obstruction of interventricular pores; tumor oppression chiasm, optic nerve caused visual impairment; tumor compression hypothalamus, The hypothalamic-pituitary dysfunction caused by the pituitary gland; the neurological and psychiatric symptoms caused by tumor invasion and other brain tissues have the following five aspects:

1. Increased intracranial pressure

The volume of craniopharyngioma is large. As an intracranial space-occupying lesion, it can directly increase the intracranial pressure through the mass effect. The craniopharyngioma can also compress the third ventricle, block the interventricular pore and make the intracranial Increased pressure, which may be the main cause of high intracranial pressure, symptoms of increased intracranial pressure are more common in children, the most common manifestation is headache, can be light and heavy, more than early morning, accompanied by vomiting, tinnitus, Dizziness, photophobia, optic disc edema, nerve palsy, etc., may also have fever, facial flushing, sweating and other manifestations of autonomic dysfunction, headaches are mostly located in the sputum, but also diffuse and to the back neck, back radiation.

Before the children's sutures are closed, the sutures are separated, the head circumference is enlarged, the sniper is broken, the scalp veins are swelled, etc., most of the patients with intracranial hypertension are larger cysts, and the tumors are forced to the third ventricle, the obstruction chamber Interstitial pores can also cause obstructive hydrocephalus. Because the internal pressure of the cyst can be changed by itself, sometimes the symptoms of intracranial hypertension are automatically relieved. Occasionally, the intratumoral cyst is ruptured, and the leakage of cyst fluid into the subarachnoid space can cause chemical meningitis. And arachnoiditis, manifested as sudden sudden headache, vomiting, with meningeal irritation, such as neck resistance, Kening sign positive, leukocytosis in the cerebrospinal fluid, fever, etc., late increased intracranial hypertension can cause coma.

2. Optic nerve compression performance

The manifestations include visual acuity, visual field changes and fundus changes. The saddle-type tumors have different compression points due to their different growth directions, which make the visual field defects very variable, which can be quadrant defects, hemianopia, dark spots, etc. Can cause visual field defects, the common side is unilateral hemianopia, such as seeing the lower quadrant unilateral hemianopia, suggesting that the compression from top to bottom, the degree of damage on both sides can be inconsistent, such as the tumor only oppresses one side of the beam, the same To hemianopia, if the tumor is severely oppressive, the primary optic atrophy may occur; if the tumor invades the third ventricle, causing hydrocephalus and increased intracranial pressure, secondary optic atrophy may occur, and the motor nerve may be involved. Symptoms such as double vision, saddle-type tumors from the bottom to the oppression of the optic chiasm, resulting in visual field defects and pituitary tumors, vision loss and optic atrophy, sometimes due to bleeding at infarction at the intersection of the intersection, blood circulation disorders caused sudden blindness, there are Optic disc edema is rare in people with optic atrophy. Foster-Kennedy syndrome can occur when the tumor grows to one side, and children have early vision defects. Many do not attract attention until it was discovered only when severe vision disorders.

3. Hypothalamus symptoms

Craniopharyngioma compression of the hypothalamus and pituitary can also cause a variety of endocrine and metabolic disorders and hypothalamic dysfunction: tumor destruction of the supraoptic nucleus or neurohypophysis, can cause diabetes insipidus, the incidence of about 20%; tumor invasion of the hypothalamus The thirst center can cause the patient to have polydipsia and polydipsia or thirst; tumor invasion and satiety center can cause polyphagia or anorexia; tumor invasion and thermoregulatory center can cause fever; tumor damage and pituitary portal system or direct Invasion of the pituitary gland can cause hypopituitarism, tumor destruction of the hypothalamic TRH, CRH, GnRH neurons can cause TSH, ACTH and gonadotropin deficiency; tumor damage and hypothalamic inhibitory neurons can cause pituitary function Hyperthyroidism, common manifestations of sexual precocity, acromegaly, deepening of skin pigmentation, hypercortisolism, etc.; some patients have obesity, lethargy, mental disorders, vasomotor dysfunction and other symptoms.

(1) Obesity-induced reproductive incompetence syndrome: the management function and reproductive activity of the nodules in the hypothalamus, and is completed by the gonadotropin in the anterior pituitary; the funnel and the gray nodules are related to fat metabolism. Compression and destruction, clinical can produce obesity, children's sexual organs are not developed, adult sexual desire disappears, women stop menstruation, lactation disorders, secondary sexual characteristics disappear.

(2) abnormal body temperature regulation: the clinical damage of the posterior hypothalamus is characterized by low body temperature (35 ~ 36 ° C), a small number of patients may have chills; the front of the hypothalamus can cause central hyperthermia (39 ~ 40 ° C ).

(3) Diabetes insipidus: manifested as increased urine output, up to several thousand milliliters or even 10,000ml per day, so a large amount of drinking water, children at night easy to trampoline, diabetes insipidus causes tumor damage, supraoptic nucleus, paraventricular nucleus, hypothalamus - Pituitary bundle or neurohypophysis causes decreased or lack of secretion of antidiuretic hormone (ADH), but polyuria is associated with normal secretion of ACTH. If the anterior pituitary is damaged at the same time, ACTH secretion is reduced, so there is no urine collapse, sometimes due to If the hypothalamic thirst center is destroyed at the same time, it can produce diabetes insipidus with thirst sensation syndrome. Although the patient has a urine collapse and high plasma permeability, there is no thirst. The urine osmotic pressure does not rise or rise slightly when the drink is banned. , blood volume reduction, hypernatremia, patients can produce headaches, tachycardia, irritability, confusion, paralysis and even coma, sometimes can produce episodes of hypotension.

(4) lethargy: seen in advanced cases, light can still wake up, heavy people sleep all day long.

(5) Psychiatric symptoms: such as forgetfulness, inattention, fiction, etc., related to the damage of the hypothalamic-marginal system or the hypothalamic frontal lobe, more common in adults.

(6) bulimia or refusal to eat: the central nervous system of the hypothalamic nucleus may have bulimia (patient obesity), and the eclipse of the ventrolateral nucleus may have anorexia or refusal (patient wasting), clinical comparison Rarely seen.

(7) Hyperprolactin (PRL): In a small number of cases, the tumor affects the hypothalamus or pituitary stalk, resulting in decreased secretion of prolactin inhibitor (PIF), increased secretion of PRL cells in the anterior pituitary, and clinically produced galactorrhea-menopausal syndrome.

(8) promote the loss of pituitary hormone secretion: hypothalamic effects can lead to loss of GHRH, TRH, CRH secretion, clinical manifestations of growth and thyroid, adrenal dysfunction.

4. Pituitary dysfunction symptoms

Pituitary dysfunction is more common than pituitary hyperfunction, especially LH/FSH and GH deficiency. It is reported that about 50% of children have delayed growth, and about 10% of children have obvious short stature with sexual dysplasia. The GH deficiency in adult patients is not prominent, but there are more than 30% of sexual dysfunction. Secondary hypothyroidism caused by insufficient TSH is found in about 1/4 of patients. Secondary adrenal insufficiency caused by ACTH deficiency is also Not uncommon.

The early manifestations of pituitary dysfunction in children are characterized by physical retardation, short stature, thinness, fatigue, fatigue, decreased activity, smooth and pale skin, yellow complexion and wrinkles, seemingly old, teeth and bones stop developing, and bones are not united. Or postponed joint, the sexual organs are infant-type, no secondary sexual characteristics, there are also manifestations of non-testosterone, a few may be afraid of cold, mild mucous edema, low blood pressure, even Simmond cachexia, adult women have menstruation Disorders or menopause, infertility and premature aging, men have decreased libido, hair loss, low blood pressure, low metabolism (up to 35%).

5. Adjacent symptoms

Tumors can grow around, such as growing to the sides, invading the sacral leaves, can cause temporal lobe epilepsy, tumors spread downward, invading the brain, can produce spastic hemiplegia, and even go to the state of brain tonic, some patients can appear spirit Abnormalities, manifested as memory loss or even loss, emotional apathy, severe confusion or dementia, such as the growth of the cavernous sinus syndrome, causing III, IV, VI on the cranial nerve disorders; sphenoid sinus, ethmoid sinus Growth can cause nosebleeds, cerebrospinal fluid rhinorrhea, etc.; growth to the anterior cranial fossa can produce mental symptoms, such as memory loss, poor orientation, can not take care of themselves, as well as epilepsy, olfactory disorders, etc.; It produces psychiatric symptoms such as temporal lobe epilepsy and illusion, phantom and odor; in a small number of patients, tumors can grow backwards to produce brainstem symptoms, and even grow to the posterior cranial fossa causing cerebellar symptoms. A small number of patients may also be affected by olfactory nerves and facial nerves. For the loss of smell and facial paralysis.

The above symptoms are slightly different in children and young patients and adult patients. The first symptoms are more common in intracranial hypertension, and the latter are more common in optic nerve compression. All patients may have endocrine changes, but adults find Earlier.

Examination of craniopharyngioma

Laboratory inspection

Ordinary laboratory tests are not special, endocrine function tests in most patients may have a low or flat delay in glucose tolerance curve, blood T3, T4, FSH, LH, GH and other hormones decreased, a few manifestations of hyperpituitarism, most performance The dysfunction of the pituitary gland and the corresponding target gland are not equal.

1. Growth hormone (GH) measurement and GH stimulation test Children with craniopharyngioma have lower serum GH value, and have no significant increase in insulin hypoglycemia, arginine, levodopa and other excitatory tests, accounting for 66.7%.

2. Gonadotropin (GnH) urinary gonadotropin (FSH), luteinizing hormone (LH) and GnH stimulation test, serum FSH, LH levels in patients with craniopharyngioma, and gonadotropin-releasing hormone (usually used For the LH-RH) stimulation test, there was no significant increase in response, suggesting that the tumor invaded the hypothalamic-pituitary region.

3. Prolactin (PRL) determination of patients with serum PRL levels can be increased, this may be due to tumor blocking prolactin release inhibitory hormone (PIH) into the pituitary, resulting in increased secretion and release of PRL, can cause galactorrhea, amenorrhea, accounting for 50%.

4. Adrenal cortex hormone ACTH, thyroid stimulating hormone TSH determination When the tumor is severely compressed by pituitary tissue and atrophy, the patient's serum ACTH, TSH decreased.

5. Antidiuretic hormone (ADH) determination of serum ADH in patients with craniopharyngioma often decreased.

6. Lumbar puncture

If there is an increase in intracranial pressure, there may be an increase in lumbar puncture pressure measurement, and there is no significant change in cerebrospinal fluid test.

Film degree exam

1. 80% to 90% of patients with skull X-ray plain film have abnormal changes in the X-ray film of the head. 94% of children with abnormal changes in the flat film, 60% of adults, the main abnormalities are as follows.

(1) Tumor calcification: There are various forms of calcification of craniopharyngioma, which are prominent features of craniopharyngioma. Both saddle-type and saddle-type tumors have calcification, while other saddle lesions rarely have calcification (the incidence of calcification). More than 1%), calcification is more common in children than in adults, the incidence of calcification in children with craniopharyngioma is 70% to 85%, 20% in children under 2 years old, 80% in children over 2 years old, 15 years old 50% of the above, about 35% of adults, when children in the calcification of the saddle, should be considered as a craniopharyngioma, calcification can be large or small, can be dispersed, can also be concentrated together, sometimes can be curved thin line Calcification often occurs in the midline area. Occasionally, larger lesions can be limited to the surrounding part of calcification. 60% to 81% of patients have tumor calcification spots, which are single or scattered, and can also be fused into eggshell.

(2) Sella change: Children with TSH and GH deficiency, bone X-ray film can show bone age reduction, the vast majority of craniopharyngioma is located in the upper part of the sella, can press the saddle down, so on the skull flat It can be found that the sella is flattened and the bed is damaged. A few craniopharyngioma are located in the saddle. The sella can be seen on the flat slice. In fact, any type of sella can be seen in the craniopharyngioma. It can be typical. The tumor on the saddle can also be changed in the saddle. In 35% of the patients, the saddle is enlarged or destroyed by a basin or a sphere. The posterior bed and saddle can be sharpened, decalcified, disappeared, and the saddle has obvious changes. Often, there are huge lesions, and vice versa.

(3) Signs of increased intracranial pressure: 60% of patients showed signs of increased intracranial pressure on the X-ray film of the skull, which showed decalcification of the saddle back, obvious cerebral gyrus in the skull, and equal manifestation of the skull base. There are skull joints and so on.

2. CT scan

CT scan of the brain showed a change in the tumor of the sellar region. The non-enhanced scan showed a high-density or iso-density image of the tumor. The calcification plaque was high-density. The cystic person showed a low-density image due to cholesterol in the tumor. The CT value was -4010Hu, the wall of the capsule is of equal density, the boundary of the lesion is clear, round, ovoid or lobulated, and the lateral ventricles are enlarged on both sides. About 2/3 of the intensive scans may have different degrees of enhancement. CT value increased by 12 ~ 14Hu, cystic craniopharyngioma showed ring enhancement or multi-ring enhancement and no enhancement in central low-density area, a few craniopharyngioma did not strengthen, generally have calcification, cystic cavity and post-enhancement enhancement The manifested saddle area tumor can be diagnosed as a craniopharyngioma (Figure 1, 2).

3.MRI

Most of the cystic parts of craniopharyngioma contain short T1 and long T2, but can also be long T1 and long T2 images, ie, low signal on T1 weighted image and high signal on T2 weighted image; Sexual craniopharyngioma is long T1 and long T2, and calcification is a low signal area.

CT and MRI examinations are important for diagnosis. These two examinations can show the location and size of the tumor, the presence or absence of cystic changes, the invasion of the tumor to adjacent brain tissue, and the presence of hydrocephalus. In general, MRI is on display. The structure of the tumor and its relationship to adjacent brain tissue (such as optic chiasm) take precedence over CT, but he cannot show calcifications like CT.

4. Ventricular angiography

Because of the different tumor sizes, ventriculography can clearly show the influence and changes of the tumor on the skull base artery. It is very helpful for surgery. Whether the tumor capsule communicates with the ventricle can be directly penetrated into the tumor capsule while performing ventriculography. .

5. EEG

The frontal or wide wave or wave is dominant.

6. Cerebral angiography shows the displacement of blood vessels in different directions due to the compression of the cerebral blood vessels by the tumor. The main sign of cerebral angiography on the saddle or from the saddle to the saddle is the upward and backward shift of the anterior cerebral artery, backward The growing tumor can compress the basilar artery and shift it backward. When the tumor grows into the third ventricle, there is a hydrocephalus-like vascular change, that is, the lateral fissure artery shifts outward, and the anterior cerebral artery moves vertically upward.

Diagnosis and diagnosis of craniopharyngioma

diagnosis

Patients with any age, such as high intracranial pressure, neurological ophthalmology and hypothalamic-pituitary dysfunction should consider the possibility of craniopharyngioma. It is not difficult to diagnose craniopharyngioma based on the location, clinical manifestations and auxiliary examination. All adolescents and children with endocrine dysfunction, such as stunting, polydipsia, obesity, genital dysplasia, etc., should first consider the disease; if there are saddle or saddle calcification, it is more helpful for diagnosis. If an adult has sexual dysfunction or headache, vision and visual impairment, this disease should also be considered.

A small number of patients with atypical clinical manifestations and mild clinical symptoms are not easy to diagnose. The key is to improve the vigilance of this disease. It is of great significance for diagnosis through laboratory examination, CT and MRI. Such examination should be done in time for suspected cases to avoid delay in diagnosis.

Differential diagnosis

(a) pituitary adenoma

1. Suspected color pituitary adenoma chromophobe pituitary adenoma is a tumor derived from the pituitary gland chromophobe, most of which break through the saddle to the saddle, clinically manifested as endocrine disorders, visual acuity, visual field changes, saddle Changes and headaches, which are similar to craniopharyngioma in clinical manifestations, but occur in adults, whose endocrine disorders are characterized by loss of libido, menopause, obesity, etc., the papillary is atrophic, often with temporal hemianopia And the destruction of the sella, patients generally do not have increased intracranial pressure, X-ray plain film examination of the saddle and saddle without calcification.

2. Eosinophilic pituitary adenoma Eosinophilic pituitary adenoma is derived from pituitary eosinophils (alpha cells), which can break through the saddle to develop oppressive optic fibers on the saddle, resulting in changes in visual acuity and visual field, due to the massive secretion of growth hormone by eosinophils. Therefore, the difference with craniopharyngioma is: 1 acromegaly occurs in adults due to osteophyte healing, clinical manifestations of hand and foot hypertrophy, mandibular protrusion, nasal enlargement, tongue widening and often hunchback, while internal organs Also hypertrophy, the patient's face is rough, the voice becomes thicker, 2 in the case of children and young people, because the callus has not healed, it is manifested as giant disease, but the development of all parts of the body is even.

(B) vertebral glioma

The glioma of the optic chiasm originates from the optic chiasm, the optic nerve and the glial cells in the hypothalamus, etc., which are more common in astrocytes, more common in adolescents, and the glioma originating from the hypothalamus can develop downward. Optic nerve or optic chiasm, glioma originating from optic chiasm can invade the thalamus upwards, so they can interact with each other (the glioma occurring in the hypothalamus and in the optic chiasm), which is clinically characterized by headache and visual field of vision. Changes, endocrine disorders and hypothalamic symptoms, headaches are mostly located in the frontal sac, often the first symptom, accompanied by nausea and vomiting, tumor invasion of the hypothalamus and pituitary, patients will have amenorrhea, loss of libido, polydipsia, vision loss The degree of bilateral divergence is often inconsistent. The visual field changes are mostly bilateral hemianopia. The same direction hemianopia and one eye blindness are also common in the visual field partial defect. The optic nerve head is mostly primary atrophy. In addition, patients can often have Drowsiness and obesity, X-ray plain film often found that the sella is enlarged, and some patients have calcification spots on the saddle, so it is sometimes difficult to identify with craniopharyngioma. But if intracranial tumors orbital type, the patients often exophthalmos identification easier.

(three) saddle nodule meningioma

The main clinical manifestations of this disease are headache, visual impairment, hypofunction of the hypothalamic hypothalamic and increased intracranial pressure. The visual acuity is slow and progressive, and it is also a common symptom of the patient. The patient has asymmetry in both eyes. The visual field of vision is missing, or one eye is blind and the other eye is normal, or one eye is blind and the other is unilateral blindness. The optic nerve head is mostly primary atrophy. A few patients have endocrine symptoms until the late stage, such as impotence, amenorrhea, etc. Light, mostly in the frontal sac, in addition to patients with olfactory dysfunction or disappearance and III, V cranial nerve dysfunction, X skull flat film can show saddle nodular saddle anterior wall bone hyperplasia, the saddle generally does not expand, Cerebral angiography shows elevation of the anterior cerebral artery. Sometimes, the outline of the tumor composed of microvessels or the radial vascular shadow centered on the saddle nodule can be seen outside the saddle nodule. Therefore, the temporal hemianopia and the optic nerve are primary. Patients with atrophy and no abnormalities in the sella can be considered as saddle nodule meningiomas.

(4) Third ventricle tumor

The incidence of children and young people is more, the tumor can block the circulation of cerebrospinal fluid and compress the structure around the third ventricle, and the corresponding clinical symptoms appear. The typical manifestations are increased intracranial pressure, paroxysmal headache and disturbance of consciousness, and lateral growth. The tumor can compress the optic tract and cause vision loss and visual field defect. The patient's paroxysmal headache is closely related to the body position. When the patient is supine, it is easy to cause seizure. When the tumor invades the upper colliculus, obesity, lethargy or urine collapse can occur, and the skull is X-ray. The calcification of the pineal gland was observed, the sella was normal, there was no pathological calcification on the saddle, and the ventricle angiography showed a third ventricle filling defect or only one side of the lateral ventricle developed and enlarged, but no displacement, and craniopharyngioma Not difficult to identify.

(5) Lateral ventricle choroidal papilloma

Choroidal papilloma often occurs in the lateral ventricle triangle. Because the tumor secretes a large amount of cerebrospinal fluid and traffic hydrocephalus occurs, the tumor can float in the ventricle, block the cerebrospinal fluid circulation pathway and cause sudden intracranial pressure rise. For severe headache, accompanied by nausea and vomiting, and even dizziness or coma, the patient often presents with a compulsive head position; due to the tumor's compression of the surrounding tissue, hemiplegia often occurs, and the lateral sensory disturbance, due to hemianopia, etc., may sometimes Hearing changes and cerebellar signs appear. Ventricular angiography shows enlargement of the lateral ventricles, displacement or filling defects. Sometimes the tumor in the lateral chamber triangle may have calcification, the pressure is widened during lumbar puncture, and the cerebrospinal fluid contains protein.

(6) saddle chondroma

The intracranial chondroma occurs in the dura mater at the base of the skull. It is generally believed that the residual chondrocytes from the outer cranial suture are developed. The patients with saddle chondroma have no increased intracranial pressure, and the clinical manifestations are eyeball protrusion and eye. De-pain, oculomotor nerve paralysis, visual impairment and visual field defects, fundus examination of the optic nerve is primary atrophy, common calcification on the skull of the skull, cerebral angiography has the internal carotid siphon segment being displaced and deformed, the disease is rare Pituitary symptoms appear.

(7) Saddle ectopic pineal tumor

Most of the ectopic pineal tumors in the saddle are gliomas, which are mostly caused by children and young people. The first symptom of most patients is diabetes insipidus, which is characterized by polydipsia and polyuria. The daily urine volume is 3000~5000ml, even up to 10000ml. Later, visual impairment and visual field defects (mostly bilateral hemianopia) gradually appeared. For example, one eye blindness and the other side of the eyelid side hemianopia and the same direction hemianopia, etc., the fundus examination of the optic nerve nipple showed primary atrophy, the patient may have hypopituitarism, loss of libido In childhood, the onset of symptoms is short stature, some have precocious puberty symptoms, a few patients have fever, respiratory changes, headaches are mostly in the forehead, more than half are accompanied by nausea and vomiting, and some patients may have III, VI cranial nerve function Obstruction, the X-ray skull was normal in the flat-skinned saddle, and there was no calcification on the saddle. The angiography showed signs of lesions on the saddle.

(8) Saddle area cholesteatoma

Intracranial cholesteat is developed from the ectodermal skin tissue remaining in the embryonic period. The saddle area is one of the most common sites. The cholesteatoma located on the saddle oppresses the optic fibers, causing vision loss and visual field defects. The optic nerve is primary. Sexual atrophy, X-ray plain film shows enlargement of the sella, supracondylar fissure, optic nerve hole, bone exudation at the anterior bed, cholesteatoma located in the saddle sometimes involving the trigeminal nerve, symptoms of trigeminal neuralgia, under the microscope The outer layer of cholesteatoma is connective tissue, and the inner wall is stratified squamous epithelium and exfoliated keratin and arranged in layers to differentiate from the craniopharyngioma.

(9) Empty Sella

Empty sellars refers to the enlargement of the saddle hole or the disappearance of the saddle. The saddle is empty and filled with cerebrospinal fluid. The pituitary atrophy and is on one side. There are primary and secondary types. The former has no obvious intracranial causes. May be due to congenital saddle hole width or saddle loss, the arachnoid sag into the saddle cavity, occupying most of it, and pressing the pituitary on one side, clinically manifested as headache, pituitary dysfunction, visual impairment Some patients may have visual impairment, and gas cerebral angiography shows enlargement of the sella. Secondary airborne sella may occur after surgical resection or radiotherapy of pituitary tumors or pituitary necrosis caused by other causes. It is basically the same as the primary one, but the visual impairment is more prominent. Sometimes the pituitary tumor can be accompanied by empty sella sphenata. The gas cerebral angiography shows that the gas is filled with the saddle cavity, accompanied by endocrine disorders, and the saddle hole can be seen during surgery. Abnormally large.

(10) temporal lobe tumor

The temporal lobe tumor is common in young people. It is more common in meningiomas and gliomas. It is mainly manifested in visual field changes, sensory aphasia, seizures, and temporal lobe tumors may occur due to compression of visual radiation or optic tract. Visual field defect or homonymous hemianopia in the same quadrant, the posterior part of the sacral middle is the sensory language center. In this area, the sensory aphasia often occurs. The patient can speak, but often mistakes, can't understand others. The meaning of speech, sometimes patients may have aphasia, there are many different hallucinations before seizures, such as hallucinations and auditory hallucinations. When the tumor compresses the internal capsule and the limbs, hemiplegia occurs. Individual patients may have a total of The cerebral angiography shows that the anterior temporal lobe tumor has a "V" shape image of the anterior cerebral artery and the middle cerebral artery, and the lateral position is like the middle cerebral artery. The anterior superior displacement, the anterior cerebral artery of the temporal lobe of the temporal lobe is shifted to the contralateral side, but the common U-shaped image of the anterior cerebral artery and the middle cerebral artery, the lateral fissure and the upper part of the bed.

(11) occipital lobe tumor

In occipital lobe tumors, there are mainly visual loss and visual field defects in the clinic. It is characterized by hemianopia or quadrant hemianopia on the contralateral side of the lesion. At the same time, there is visual perception and illusion, and the illusion is not formed, such as lightning, Mars or Circles, etc., and can float up and down, hallucinations can also be a precursor to epilepsy. There is a head-eye rotation center at the junction of the top pillow. When this area is affected, the head eye rotates to the opposite side of the lesion, which is easy to identify with the craniopharyngioma.

(12) sphenoid ridge meningioma

The need to identify with craniopharyngioma refers to a meningioma that occurs in 1/3 of the sphenoid ridge. The sphenoid ridge is a good site for meningiomas, and the incidence of 1/3 is about 25%. Clinically, The performance is visual field defect, eye movement disorder, pupil dilation, slow response to light and drooping eyelids, etc. This is caused by oculomotor nerve, optic nerve and supracondylar fissure. In addition, patients often have olfactory loss, a small number of patients There was a hemiplegia of the contralateral limb. The fundus examination revealed a primary atrophy of the lateral optic nerve, and edema of the lateral papillary sac. The flat slice of the skull showed a decrease in the bone density of the sacral wall of the lesion, an enlarged sacral fissure and a blurred outline, and an abnormality of the optic nerve. The cerebral angiography showed a slight shift of the proximal middle cerebral artery to the posterior superior part of the middle cerebral artery. The intracranial epidural segment of the internal carotid artery was displaced backward and the siphon segment was opened.

(13) Saddle area germ cell tumor

That is, ectopic pineal tumor, accounting for the fourth place, 70% of patients are aged between 7 and 20 years old, and often have endocrine disorders, but with diabetes insipidus as a prominent symptom, may be associated with precocious puberty, may also have vision, vision Change, the saddle is normal.

(14) Epidermoid cysts in the sellar region

Rarely, the majority of the onset age is between 23 and 37 years old, with visual acuity and visual field disorder as the main manifestations, generally no endocrine disorders, symptoms of increased intracranial pressure are rare, normal saddle, no calcification, CT scan In the low-density lesions in the Saddle area, the CT values were mostly negative and not enhanced.

(15) Chordoma

It occurs mostly in the age of 35, with multiple cranial nerve injuries as the main manifestation, often with calcification, obvious bone destruction in the saddle and slope, CT shows irregularly high density lumps, of which there are calcifications, most do not occur Strengthened, a few can have even light reinforcement.

(16) Aneurysms in the sellar region

Rare, more common in middle-aged people, characterized by sudden onset, headache, oculomotor nerve paralysis, the saddle generally no change, cerebral angiography can be diagnosed, intraoperative puncture is blood, the tumor does not collapse.

(17) Vision cross arachnoiditis

More common in adults, with visual acuity, visual field changes as the main performance, visual field changes are generally irregular, irregular changes, visual field reduction, generally no endocrine disorders and increased intracranial pressure, normal saddle, CT scan of the saddle-free space-occupying lesions .

(18) Saddle area arachnoid cyst

Rare, more common in children, can also be seen in adults, the main symptoms are hydrocephalus caused by hydrocephalus, may have vision, visual field changes, a small number of patients with endocrine symptoms, saddle enlargement or double saddle bottom, CT scan see cerebrospinal fluid density Round low density area.

The identification of craniopharyngioma and the above diseases mainly depends on the following two points: in addition to the above-mentioned diseases, similar to those of craniopharyngioma, there are other manifestations that do not conform to craniopharyngioma; the above diseases and craniopharyngioma Tumors are different in imaging. Generally speaking, by CT and MRI, combined with clinical manifestations, the vast majority of cases can be clearly diagnosed. Very few cases may need to rely on the pathological examination of surgical specimens to confirm the diagnosis, with high intracranial pressure. The main manifestations of craniopharyngioma need to be differentiated from other intracranial space-occupying lesions; visual acuity, visual field changes and other saddle lesions such as pituitary adenoma, optic glioma, sellar meningioma, internal carotid aneurysm and Differentiation of embryonic tissue tumors, because these saddle lesions can also damage the optic chiasm, optic nerve and optic tract to cause similar symptoms; the main manifestations of hypopituitarism need to be differentiated from other diseases that cause hypopituitarism.

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