Origin of craniopharyngiomas: implications for growth pattern, clinical characteristics, and outcomes of tumor recurrence

Abstract

C raniopharyngioma, representing 1.2%-4.4% of CNS tumors, can still be challenging. Although its histology is benign, this tumor is associated with a high rate of recurrence. 22 Recurrent craniopharyngiomas are associated with high morbidity because of the tumor's proximity to vital structures, including the pituitary stalk, hypothalamus, optic apparatus, and vessels in the circle of Willis.Various strategies have been advocated to improve patient outcomes. Total resection of recurrent craniopharyngioma remains a challenge even in experienced hands, particularly if the lesion involves the hypothalamus and the third ventricle, because of the associated risk of hypothalamic injury. 20 Loss of arachnoid interposed between the craniopharyngioma and the surrounding hypothalamus has been proposed as a main reason for the higher abbreviatioNs ACTH = adrenocorticotropic hormone; BMI = body mass index; DI = diabetes insipidus; FSH = follicle-stimulating hormone; GH = growth hormone; KPS = Karnofsky Performance Scale; LH = luteinizing hormone; TSH = thyroid-stimulating hormone. Department of Neurosurgery, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong, People's Republic of China obJective Craniopharyngiomas are associated with a high rate of recurrence. The surgical management of recurrent lesions has been among the most challenging neurosurgical procedures because of the craniopharyngioma's complex topographical relationship with surrounding structures. The aim of this study was to define the determinative role of the site of origin on the growth pattern and clinical features of recurrent craniopharyngiomas. methods The authors performed a retrospective analysis of 52 patients who had undergone uniform treatment by a single surgeon. For each patient, data concerning symptoms and signs, imaging features, hypothalamic-pituitary function, and recurrence-free survival rate were collected. results For children, delayed puberty was more frequent in the group with Type I (infradiaphragmatic) craniopharyngioma than in the group with Type TS (tuberoinfundibular and suprasellar extraventricular) lesions (p < 0.05). For adults, blindness was more frequent in the Type I group than in the Type TS group (p < 0.05). Nausea or vomiting, delayed puberty, and growth retardation were more frequent in children than in adults (p < 0.05). Overall clinical outcome was good in 48.07% of the patients and poor in 51.92%. Patients with Type TS recurrent tumors had significantly worse functional outcomes and hypothalamic function than patients with the Type I recurrent tumors but better pituitary function especially in children. coNclusioNs The origin of recurrent craniopharyngiomas significantly affected the symptoms, signs, functional outcomes, and hypothalamic-pituitary functions of patients undergoing repeated surgery. Differences in tumor growth patterns and site of origin should be considered when one is comparing outcomes and survival across treatment paradigms in patients with recurrent craniopharyngiomas.

Craniopharyngioma recurrence: the impact of tumor topography

“…These adhesions will happen predominantly within the basal hypothalamus for infundibulo-tuberal lesions and underneath the diaphragma sellae, at the dorsal aspect of the hypophyseal gland for sellar and/or suprasellar tumors. This concept of a close relation between the recurrence rate and the original topography of CPs is very much supported by the finding of coincidental locations for primary and recurrent CPs in the study by Bao et al 1 To conclude, contrary to the general belief that recurrent CP surgery is associated with higher morbidity and mortality rates given the difficulties posed by the scarring process induced during prior procedures, one should remember that the likelihood of a successful outcome following radical removal of a recurrence may largely depend on their original topography. The quality of life of patients with these lesions is mostly related to the preservation of hypothalamic functions.…”

“…Apart from their results, we wish to highlight the importance of their analysis, as they found that, among the potential factors that influence patient outcome following repeated surgery for CPs, tumor topography is a major variable that predicts both the postoperative status and the hypothalamic functional outcome. 1 Comparison of recurrent CPs growing below (15 tumors) or above (37 tumors) the diaphragma sellae revealed that an acceptable outcome occurred in 87% of the infradiaphragmatic cases, whereas a good outcome was achieved in only 32% of the supradiaphragmatic tumors.…”

“…27 Bao et al observed more extensive adhesions as well as a worse outcome among recurrent CPs not covered by the diaphragma sellae and in close relation with the third ventricle. 1 Consequently, although the resectability of recurrent CPs rests on the surgeon's subjective judgment of the feasibility of removing tenacious adherences between tumor and neighboring structures, it is the objective damage inflicted on vital brain structures that will eventually determine the surgical outcome. Patient outcome following surgery for recurrent CPs has been found to depend mainly on the hypothalamic injury caused by surgical manipulation, 1,8,12 as was evidenced by early attempts at removing primary CPs.…”

“…1 Consequently, although the resectability of recurrent CPs rests on the surgeon's subjective judgment of the feasibility of removing tenacious adherences between tumor and neighboring structures, it is the objective damage inflicted on vital brain structures that will eventually determine the surgical outcome. Patient outcome following surgery for recurrent CPs has been found to depend mainly on the hypothalamic injury caused by surgical manipulation, 1,8,12 as was evidenced by early attempts at removing primary CPs. 20 In the particular case of recurrent CPs involving the third ventricle, the worst surgical outcome has been related to the loss of the gliotic envelope associated with previous surgical maneuvers.…”

Intraventricular craniopharyngioma: a case report

“…If these cells develop into a tumor, it will be a purely intraventricular craniopharyngioma. 5 Intraventricular craniopharyngiomas (IVCP) are rare, both in infants and adults, 1 but they are usually diagnosed in older patients. Two reasons explain this: low rate of tumor replication and location in the third ventricle.…”