What do you know about craniopharyngioma?

  Recently, I have treated several cases of craniopharyngioma. The patient’s family asked me when we met, “Can you guarantee complete resection of the tumor? Many people in my QQ friend group have advised me to visit a hospital that guarantees total resection in every case, I will not operate on my child if you dare not guarantee it, I want to find the best doctor in the world so that my child will never recur again ……” Although the parents’ words are radical, they represent the voice of all patients’ families: how should we choose between total removal of craniopharyngioma without preserving the body’s function or keeping the tumor that cannot be removed locally so that the patient can live a normal life?  Craniopharyngioma is an intracranial tumor that is very similar to pituitary tumor and can cause hypothalamic pituitary dysfunction, increased intracranial pressure, visual and visual field disorders, uveitis, and neurological and psychiatric symptoms to patients.  Craniopharyngioma is a congenital disease with slow growth. During normal embryonic development, the elongated canal that connects the Rathke’s bursa to the primitive oral cavity, the craniopharyngeal duct, gradually disappears as the embryo develops, and the remnants of the anterior wall of the Rathke’s bursa, the anterior lobe nodules, and the remaining squamous epithelial cells of the degenerated craniopharyngeal duct may be the origin of craniopharyngioma. Thus, craniopharyngiomas can occur in the pharynx, pterygoid sinus, intra- and supra-saddle, and third ventricle, and some can invade the posterior cranial fossa. Most craniopharyngiomas originate from the residual squamous epithelial cells in the craniobuccal canal near the funnel, so the tumor is located in the suprasellar region, forming the so-called “suprasellar” type of craniopharyngioma. The tumor is located in the saddle, forming the so-called “suprasellar” craniopharyngioma. Some craniopharyngiomas are found in both the suprasellar and intersellar regions, and then the tumor is dumbbell-shaped.  The degree of resection of craniopharyngioma is very controversial. The emphasis on total resection of tumor is inevitably accompanied by high mortality and disability rate; the advocate of subtotal resection plus postoperative radiotherapy faces a high recurrence rate after surgery. Intraoperatively, it was found that in many cases, the tumor was closely adhered to the surrounding important structures such as internal carotid artery, hypothalamus, optic nerve and optic cross, and reluctant resection would cause direct or indirect damage to these structures, such as internal carotid artery spasm ischemia and hypothalamic injury were the main causes of postoperative death and disability. Among the patients whose tumors were not completely resected, it was because the tumors were closely adherent to the hypothalamus, closely related to the pituitary stalk, adherent to the internal carotid artery, and adherent to the optic nerve. Among the adherent parts, calcification is the most difficult to be separated; while the part of solid craniopharyngioma adhering to hypothalamus often has a glial proliferation zone that can be used as an interface for surgical operation, thus reducing the damage to hypothalamus.  About recurrence after craniopharyngioma resection The recurrence rate 5 years after total resection of craniopharyngioma is 10%-17%, and the recurrence of craniopharyngioma is closely related to the degree of tumor resection. As for the time of tumor recurrence, most tumor recurrence occurs within 5 years and decreases significantly afterwards. Some scholars have also reported that the recurrence rate after craniopharyngioma resection via the butterfly approach is low, which may be related to the fact that craniopharyngioma using this approach is mainly confined to the saddle and has less adhesion to the surrounding important structures.  The quality of life of patients is seriously affected by the damage of craniopharyngioma surgery itself and the recurrence after surgery. The patient’s family is taking care of the child who is in a vegetative state, or the pain and helplessness of seeking medical help for postoperative urinary collapse, and it is difficult to complain, and even some doctors presume to comment that it is too much to cut. Where to go from here, there is no agreement. …… The doctor with resentment and misunderstanding is still trying to move forward, they think that let the patient have a quality of life to live is the purpose of the doctor’s treatment. The patient’s family still believes that total tumor removal is the way to go; is total tumor removal, high risk of quality of life deterioration, long-term coma and death after surgery the result we expect to accept?  Believe the surgeon who operates on you, his judgment is the result of years or even decades of experience, he will not let go of any tumor that can be cut off, and he will not do anything rashly if it cannot be reached. Partial removal or total excision has become a problem to be solved in the medical world and even in medical philosophy. Let’s calm down, contemplate and analyze, and work together to choose a middle path in the current situation where total removal is difficult and the desired result is guaranteed. Let us pray that craniopharyngioma will no longer harm human health, and let us hope that the doctor’s craft will be able to cut the tumor and save lives.