What are the symptoms of craniopharyngioma?

  Craniopharyngioma originates from flattened epithelial cells remaining during pituitary embryogenesis and is a common congenital intracranial benign tumor, mostly located above the pterygoid saddle and a few within the saddle.  1. Increased intracranial pressure manifestation: Craniopharyngioma is large in size, and as an intracranial occupying lesion, it can directly cause increased intracranial pressure through the occupying effect. Craniopharyngioma can also compress the third ventricle and obstruct the interventricular foramen, which may be the main cause of high intracranial pressure. The most common symptom of increased intracranial pressure in children is headache, which can be mild or severe, and occurs early in the morning, accompanied by vomiting, tinnitus, vertigo, photophobia, optic disc edema, spreading nerve palsy, etc. Autonomic dysfunction such as fever, facial flushing, and sweating may also be present. The headache is mostly located behind the orbit, but can also be diffuse and radiates to the back of the neck and back. In children before the sutures are closed, the sutures are separated, the head circumference is enlarged, the sound of breaking cans on percussion, and the scalp veins are angered. Most of the intracranial hypertension is caused by larger cysts, and the tumor compressing the third ventricle and obstructing the interventricular foramen may also cause obstructive hydrocephalus. Since the pressure inside the cyst can change by itself, sometimes the symptoms of intracranial hypertension appear to be relieved automatically. Occasionally, the cyst may rupture and the cystic fluid may leak into the subarachnoid space, which may cause chemical meningitis and arachnoiditis, manifested as sudden severe headache and vomiting, accompanied by symptoms of meningeal irritation, such as cervical resistance, positive Kening’s sign, increased leukocytes in cerebrospinal fluid and fever. Late stage intracranial hypertension may lead to coma.  2. Optic nerve compression manifestations: visual acuity, visual field changes and fundus changes. The suprasellar tumor has different compression sites due to its irregular growth direction, so the visual field defect varies greatly, which can be quadrant defect, partial blindness, dark spot, etc. Tumor compression on the optic cross can cause visual field defect, the common one is temporal hemianopia, if we see bilateral temporal hemianopia in the lower quadrant, it indicates that the compression is from top to bottom, and the degree of damage on both sides may not be the same. If the tumor compresses only one side of the optic tract, it will produce isotropic hemianopia. If the tumor severely compresses the optic cross, it may cause primary optic nerve atrophy; if the tumor invades the third ventricle and causes hydrocephalus and increased intracranial pressure, it may produce secondary optic nerve atrophy. Oculomotor nerve may be involved, resulting in diplopia and other symptoms. Intersaddle type tumor compresses the optic cross from the bottom to the top, producing visual field defects same as pituitary tumor, and vision loss is related to optic nerve atrophy. Sometimes, sudden blindness can be caused by hemorrhagic infarction at the optic cross and impaired blood circulation. In patients with primary optic nerve atrophy, optic disc edema rarely occurs again. Foster-Kennedy syndrome can occur when the tumor grows to one side. In children, the early visual field defects are not noticed until the visual acuity is severely impaired.  Hypothalamic symptoms: craniopharyngioma compression of hypothalamus and pituitary gland may cause various endocrine metabolic disorders and hypothalamic dysfunction: tumor damage to supraoptic nucleus or pituitary gland may cause uropathy, the incidence of which is about 20%; tumor invasion of hypothalamic thirst center may cause patients to drink a lot or lose thirst; tumor invasion of satiety center may cause hyperphagia or anorexia; tumor invasion of thermoregulatory center may cause fever; tumor damage to pituitary center may cause fever. Tumor damage to pituitary portal system or direct invasion of pituitary gland may cause hypopituitary function. Tumor damage to hypothalamic TRH, CRH and GnRH neurons may cause TSH, ACTH and gonadotropin deficiency respectively; tumor damage to hypothalamic inhibitory neurons may cause hyperpituitary function. Some patients have symptoms such as obesity, drowsiness, mental disorder and vasodilator disorder.  Pituitary dysfunction symptoms: Hypopituitarism is more common than hyperpituitarism, especially LH/FSH and GH deficiency. About 50% of pediatric patients have growth delay, and about 10% of children show significant dwarfism with sexual dysplasia. Secondary hypothyroidism due to TSH deficiency is seen in about 1/4 of patients, and secondary hypoadrenocorticism due to ACTH deficiency is not uncommon.  Early manifestations of pituitary insufficiency in children include delayed physical growth, short, thin body, fatigue, reduced activity, smooth and pale skin, yellowing and wrinkles, and an appearance of old age. The teeth and bones stop developing, the bones do not unite or delay uniting, the sexual organs are infantile, there is no secondary sexual characteristics, and there are also people who show azoospermia. A few may have cold fear, mild mucus edema, low blood pressure, or even Simmond cachexia. Adult women have menstrual disorders or menopause, infertility and premature aging. Men have decreased libido, hair loss, low blood pressure, and low metabolism (up to 35%).  5. Adjacent symptoms: The tumor may grow in all directions, such as to both sides and invade the temporal lobe, which may cause temporal lobe epilepsy. If the tumor expands downward and invades the foot of brain, it can produce spastic hemiparesis and even deactivated state. Some patients may develop mental disorders, manifesting as memory loss or even loss, emotional indifference, and in serious cases, mental blurring or dementia. If the tumor grows to the paracranial fossa, it may produce cavernous sinus syndrome, causing III, IV and VI cerebral nerve disorders; if it grows to the pterygoid sinus and sieve sinus, it may cause rhinorrhea and cerebrospinal fluid nasal leakage; if it grows to the anterior cranial fossa, it may produce psychiatric symptoms, such as memory loss, poor orientation, inability to take care of oneself, epilepsy and smell disorder; if it grows to the middle cranial fossa, it may produce temporal lobe epilepsy and psychiatric symptoms such as phantom smell and smell; in a few patients, the tumor may grow backward and produce psychiatric symptoms. In a few patients, the tumor may grow backward and produce brainstem symptoms, or even grow to the posterior cranial fossa and cause cerebellar symptoms. In a few patients, the olfactory and facial nerves may also be involved, manifesting as loss of smell and facial palsy.