Childhood craniopharyngioma: a retrospective study of children followed in Hôpital Universitaire de Bruxelles

Abstract

Introduction: Craniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients.

Methods: We performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles.

Results: Nineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery.

Conclusion: CP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.

Keywords: childhood craniopharyngioma; endocrine disorders; hypothalamic obesity; neurosurgery; radiotherapy; visual disorders.

Figure 1

Brain MRI and grading according to Müller’s classification. Grade 0. Reference from: Otte A, Müller HL. Childhood-onset Craniopharyngioma. J Clin Endocrinol Metab. 27 sept 2021;106(10):e3820−36. (A) Preoperative sagittal T1-WI shows a predominantly cystic lesion in the sella turcica and the suprasellar cistern. No mass effect is visible on the infundibulum, the floor of the third ventricle or the mamillary bodies. (B) Postoperative sagittal T1-WI. Grade 1. (C) Preoperative sagittal T2-WI shows a predominantly cystic mass of the hypothalamic-pituitary region pushing back the floor of the third ventricle (no mass effect on the mamillary bodies). (D) Postoperative sagittal T1-WI with Gadolinium shows a complete resection of the tumor (no tumor residue). Grade 2. (E) Preoperative sagittal T1-WI after contrast administration, shows a solid and cystic mass in the hypothalamo-pituitary region compressing and invading the anterior and posterior hypothalamus. (F) Postoperative sagittal T1-WI after gadolinium, shows mass effect resolution. MRI, magnetic resonance imaging; WI, weighted imaging.

Figure 2

Evolution of endocrinological deficits at diagnosis and after surgery. PO, postoperative.

Figure 3

Evolution of BMI at diagnosis and after surgery. Obesity was defined with BMI ≥ 2 SDS. Thirteen percent of patients (2/15) were already obese at the time of diagnosis. There was no statistically significant difference between the preoperative BMI and the 6-month postoperative BMI (p=0.273). BMI, body mass index; SDS, standard deviation score; NA, not applicable.

Figure 4

Evolution of median BMI and height at diagnosis and after surgery. BMI and height expressed in SDS. The postoperative occurrence of weight gain was increased at 6-months and 12-months after surgery, then reaching a plateau during the rest of the follow-up. Recombinant growth hormone replacement therapy is usually initiated between 6 months and 1 year after the completion of oncological therapy. PO, postoperative; BMI, body mass index; SDS, standard deviation score.