Craniopharyngioma is a common embryonic remnant tissue tumor that develops from the epithelial cells of the remnants of the craniopharyngeal duct formed in the ectoderm. It is the most common intracranial congenital tumor that occurs in children and less commonly in adults, most often in the suprasellar region. Its main clinical features include hypothalamic-pituitary dysfunction, increased intracranial pressure, visual acuity and visual field disorders, uveitis, and neurological and psychiatric symptoms, which can be clearly diagnosed by CT scan. Treatment is mainly surgical removal of the tumor.
The disease can develop at any age, but 70% occurs in children and adolescents under 15 years of age; the peak incidence in children is about 5-10 years of age and in adults is about 50-60 years of age. Craniopharyngioma accounts for 5%-6% of intracranial tumors; about 5%-10% of intracranial tumors in children.
Pathology】Major specimen: the tumor indicates smooth or mildly bumpy nodularity, the tumor is mostly cystic or multicystic, few are completely parenchymal, the cyst fluid is yellow or yellow-brown, coffee-colored viscous liquid, the contents are complex, including cholesterol crystals, common protein, keratin and calcified deposits. The cyst wall mostly has calcified spots. Microscopic examination: divided into enamel type and papillary type.
[Diagnosis basis
1.Clinical manifestations may appear in three groups of symptoms of different degrees.
(1) Endocrine dysfunction: caused by tumor involving pituitary and hypothalamus, including growth and development disorders, sexual dysfunction, fat, water and electrolyte metabolism disorders, mental disorders, manifesting as symptoms and signs of uropathy and hypopituitary function.
(2) Tumor compression symptoms: headache, visual field impairment, and affecting other brain nerves causing corresponding symptoms.
(3) Symptoms of increased intracranial pressure: the enlargement of tumor leads to cerebrospinal fluid circulation disorder, resulting in obstructive hydrocephalus.
2.Auxiliary examination
(1) X-ray plain film: most of the patients show abnormal calcification in saddle area and suprasellar area and some patients show signs of increased intracranial pressure.
(2) CT: The plain scan shows a low-density cystic or cystic solid mass in the saddle, with clear borders, round or oval, or lobulated lesions. The cystic wall calcification is arcuate, while the parenchymal calcification is punctate. The suprasellar pool is partially or completely closed and there may be signs of obstructive hydrocephalus. In case of cystic craniopharyngioma, the cyst wall is thinly circumscribed and the central low-density cystic fluid does not strengthen after enhancement. A small number of craniopharyngiomas do not strengthen or show uniform strengthening.
(3) MRI: T1-weighted image shows that the cystic contents of the tumor are mostly high signal, and the parenchymal part of the tumor is equal to or low signal; T2-weighted image shows that the cystic component is high signal, and the calcification is almost always low or very low signal on T2-weighted image. The parenchymal part of the tumor and the cystic wall can be enhanced after enhancement, but a significant proportion of craniopharyngiomas do not enhance.
Differential diagnosis】Differentiate from pituitary adenoma, saddle node meningioma, glioma of the optic nerve and anterior third ventricle, germ cell tumor, chordoma, epidermoid cyst in the saddle area, epithelioid cyst and aneurysm of the siphon segment of the internal carotid artery.
Treatment and prognosis
1.Surgical treatment: If the patient’s general condition is good, the preferred treatment is total resection of the tumor. If the tumor is closely adhered to the internal carotid artery, optic nerve and other surrounding tissues, and if the tumor infiltrates the hypothalamus, even if it is barely removed, the effect may not be satisfactory. There are various approaches, such as inferior frontal approach, pterygoid point approach, endplate approach, intercallosal vault approach or lateral ventricular approach, pterygoid sinus approach, combined approach, etc. Intrasellar craniopharyngioma is an indication for transsphenoidal surgical resection.
If the patient has serious hypothalamic symptoms, is bedridden and cannot tolerate craniotomy, cystic puncture can be performed or the cystic fluid can be aspirated by stereotactic technique to reduce the pressure of the tumor on the surrounding structures, and isotopes can be injected at the same time for internal irradiation treatment.
Many scholars believe that radiotherapy can increase postoperative survival rate and reduce tumor recurrence. The general radiotherapy dose is 50-60Gy.
Chemotherapy is generally not applied to systemic chemotherapy drugs, but chemotherapy drugs (such as bleomycin) can be injected into the tumor cavity through the Ommaya capsule.
4.Hormone replacement therapy is applied to patients with hypopituitarism, generally supplemented with prednisone, and the dose is decided according to the hypoplasia.
5.Prognosis The 5-year survival rate is 64?9% for surgery alone and 82?5% for those who add radiotherapy; the tumor recurrence rate is 0-30% for postoperative plus radiotherapy and 75%-78% for surgery alone.