Craniopharyngioma in Children Q&A

1. incidence of craniopharyngioma? Is there any gender difference? The incidence of craniopharyngioma has regional differences and age tendency. Craniopharyngioma accounts for 6-9% of intracranial tumors in children in Europe and America, and 12-13% in Asia. The annual incidence rate of craniopharyngioma is 1.2-5.25 per million children, with the peak of incidence at the age of 8-12 years. Most scholars believe that there is no obvious gender difference in the incidence of craniopharyngioma, while some scholars believe that there are slightly more males than females. 2.Is craniopharyngioma a benign or malignant tumor? Craniopharyngioma in children belongs to congenital tumors, which can be divided into cystic, solid and cystic-solid morphologically, and most of them are cystic-solid. The cystic part of the tumor often grows rapidly, and the condition changes rapidly, and the patient can suffer from blindness, hydrocephalus, damage of hypothalamus function, hemiparesis, coma or even death in a short period of time. Some people think that the tumor is malignant because it is easy to recur after surgery and the tumor can be seen to infiltrate the brain tissue pathologically. Most people think that craniopharyngioma is a benign tumor, but the course of the disease is malignant. 3.Why is it difficult to operate craniopharyngioma? The biggest problem of craniopharyngioma is that it occurs in a part of the body that is close to the most important structures of the human body and is closely adhered to them, such as intracranial arteries, brainstem, hypothalamus-pituitary system, and multiple cranial nerves, and the surgical field of view is narrow and deep, which makes the total resection very difficult. Even if it is partially resected, the damage of related structures is enough to be fatal, so the surgery of craniopharyngioma is full of risks and challenges for both patients and doctors, and it must be done by experienced doctors, or else the postoperative mortality rate will be greatly increased. 4.How should Craniopharyngioma be treated? Microsurgery is the first choice for the treatment of craniopharyngioma, and the choice of different surgical approaches is based on the growth site of the tumor and the surgeon’s own experience. Commonly used surgical approaches include trans-frontal base approach, frontotemporal approach (wing point approach), trans-lateral ventricle approach, trans-medial fissure endplate approach, trans-callosal inter-dome approach, and so on. 5.What are the comorbidities in the acute postoperative period? The severity of acute postoperative comorbidities depends on the degree of tumor adhesion and the surgeon’s surgical technique. Most patients will have electrolyte disorders, fever, and excessive drinking and urination. Some patients may develop seizures, hydrocephalus, hemiparesis, and subdural effusion. Craniopharyngioma surgery should be mastered, neither to pursue total excision and lead to the patient’s coma and death, nor to easily give up to do palliative care, such as placing an Omaya tube inside the tumor capsule, and suctioning at regular intervals. The highest level of surgery is definitely the highest degree of tumor resection and the least comorbidity. 6.What are the treatment options for craniopharyngioma other than surgery? Even if the tumor recurs, surgical treatment is preferred, and cure is achieved by total resection of the tumor through different accesses and multiple surgeries. If it is really difficult to cut the tumor completely after repeated efforts, radiotherapy can be chosen, which can reduce the recurrence rate and prolong the recurrence time. Gamma Knife treatment is rarely done for craniopharyngiomas unless the residual tumor is solid. Cystic residual tumor can also be treated with intracapsular radiotherapy and chemotherapy after aspiration of the cystic fluid, mainly by injection of radioisotope and bleomycin. 7.What is the survival rate of craniopharyngioma? The 5-year survival rate is 95%, the 10-year survival rate is 80-90%, and the 10-year survival rate of recurrent craniopharyngioma is 70%. 8.What are the long-term comorbidities of craniopharyngioma? Postoperative long-term comorbidities of craniopharyngioma mainly include obesity, urolithiasis and growth disorder caused by hypopituitarism. Hypopituitarism can be caused by postoperative, post radiotherapy or hydrocephalus. Symptoms include hypoglycemia, altered blood sodium concentration, shock, and growth retardation, etc. They can appear acutely after surgery or be delayed, and some of the symptoms are fatal and require long-term follow-up treatment. Complete treatment of craniopharyngiomas also requires the participation of endocrinologists, especially concerning growth and reproduction. 9.What are the long-term sequelae of craniopharyngioma? In long-term follow-up, the main symptoms include headache in 64%, visual field defect in 55%, hydrocephalus in 23%, GH, FSH/LH, ACTH, TSH deficiency in 88%, 90%, 86%, 80%, diabetes insipidus in 65%, obesity in 39%, epilepsy in 12%, and hemiplegia in 11%. The prevalence of cardiovascular, neurological and psychosocial diseases is 22%, 49% and 47% respectively. The prevalence of cardiovascular diseases was 22%, 49% and 47% respectively. In the long-term follow-up of patients, in addition to paying attention to the recurrence of tumors, the main focus is to adjust the endocrine system, especially the estrogen replacement therapy for premenopausal women, as well as controlling sugar, fat, blood pressure and body weight to reduce the risk of cardiovascular disease. Attention should be paid to pathological obesity and cerebral cortical dysfunction, and the patient’s memory, behavior, and emotional changes affect their social quality of life. Growth hormone replacement therapy is effective for height growth, but is less effective in treating obesity and has the potential to stimulate tumor recurrence.