Surgery for pediatric craniopharyngiomas: is less more?

Abstract

Surgery for craniopharyngiomas, especially in childhood and adolescence, has evolved from an era of aggressive strategies - with the primary goal of gross total removal and accepting an impaired functional outcome - to a more individually tailored therapy that avoids immediate treatment-related and long-term morbidity. Modern imaging techniques and a wider understanding of hypothalamic risk factors have led to surgical strategies adapted to the localization of these inhomogenously grown pathologies. Whereas purely infradiaphragmatic as well as supradiaphragmatic/infrachiasmatic tumors have a favorably surgical outcome with higher gross total resection rates in experienced hands, lesions within the third ventricle extending beyond the mammillary bodies remain a problem. The same is valid for lesions beyond 3 cm in diameter, more or less independent of their localization. Aside from the traditional microscopic approach via the subfrontal or pterional craniotomy, transsphenoidal approaches and other minimal invasive surgical methods, e.g., catheter implantation into cystic formations of the tumor have become popular. Radiotherapy, with its risks and limitations, can effectively be added to avoid recurrences. Nowadays, surgery as part of an interdisciplinary treatment strategy is still the typical first choice. However, taking the patient's long-term prognosis into considertaion, the surgical complication rates have to be minimized.

Gliomas, germ cell tumors, and craniopharyngioma

“…Historically, transcranial, supraorbital, or transsphenoidal approaches have been employed based upon location and extent of tumor 71,78 . Improvements in endoscopic and microsurgical resection techniques, as well as planned subtotal resection with adjuvant radiotherapy, may further diminish morbidity 77,79‐81 …”

Craniopharyngioma and hypothalamic injury

“…For favourably localized tumours, the preferred treatment of choice, especially at primary craniopharyngioma diagnosis, is an attempt at complete resection with preservation of visual and hypothalamic function [ 6 ▪ , 12 , 39 , 45 – 50 ]. For unfavourably localized tumours – those too close to or too entangled with the optic chiasm and/or the hypothalamus – a planned limited resection should be performed to preserve integrity of and/or to avoid further damage to the hypothalamic and optic structures [ 4 ▪ , 51 ▪ , 52 , 53 , 54 ▪▪ , 55 ▪ – 57 ▪ ]. Endoscopic routes provide novel and less traumatic approaches for surgical resection [ 58 ▪ , 59 ].…”

“…Associated with high morbidity, suprachiasmatic lesions are difficult to treat. Surgical removal of tumour tissue beyond the mammillary bodies risks hypothalamic structures and consequent hypothalamic obesity [ 39 , 40 , 51 ▪ , 73 ▪ ]. With the aid of imaging studies, recent reports have indicated that the degree of obesity of affected patients is positively correlated with the degree of hypothalamic damage [ 39 , 40 – 42 ▪ ].…”

“…With the aid of imaging studies, recent reports have indicated that the degree of obesity of affected patients is positively correlated with the degree of hypothalamic damage [ 39 , 40 – 42 ▪ ]. Taking these considerations into account, novel classifications of presurgical involvement and postsurgical lesions of hypothalamic structures based on MRI have been recently published [ 40 , 41 , 51 ▪ ] that might help to establish risk-adapted, that is, hypothalamus-sparing surgical strategies.…”