Management strategies for recurrent pediatric craniopharyngioma: new recommendations

Abstract

OBJECTIVE The goal of this study was to identify the independent risk factors for recurrence or progression of pediatric craniopharyngioma and to establish predictors of the appropriate timing of intervention and best management strategy in the setting of recurrence/progression, with the aim of optimizing tumor control. METHODS This is a retrospective cohort study of all pediatric patients with craniopharyngioma who were diagnosed and treated at Boston Children’s Hospital between 1990 and 2017. This study was approved by the institutional review board at Boston Children’s Hospital. All statistical analyses were performed using Stata software. RESULTS Eighty patients (43 males and 37 females) fulfilled the inclusion criteria. The mean age at the time of diagnosis was 8.6 ± 4.4 years (range 1.2–19.7 years). The mean follow-up was 10.9 ± 6.5 years (range 1.3–24.6 years). Overall, 30/80 (37.5%) patients developed recurrence/progression. The median latency to recurrence/progression was 12.75 months (range 3–108 months). Subtotal resection with no adjuvant radiotherapy (p < 0.001) and fine calcifications (p = 0.008) are independent risk factors for recurrence/progression. An increase (%) in the maximum dimension of the tumor at the time of recurrence/progression was considered a statistically significant predictor of the appropriate timing of intervention. CONCLUSIONS Based on the identified independent risk factors for tumor recurrence/progression and the predictors of appropriate timing of intervention in the setting of recurrence/progression, the authors propose an algorithm for optimal management of recurrent pediatric craniopharyngioma to increase the likelihood of tumor control.

Expanded Transsphenoidal Trans-Lamina Terminalis Approach to Tumors Extending Into the Third Ventricle: Technique Notes and a Single Institute Experience

“…Furthermore, radiotherapy to treat these tumors can also be damaging. In addition, the risk of reoperation for recurrent tumors after radiotherapy is higher, and the total resection rate can be significantly lower (31)(32)(33), especially for large cystic craniopharyngiomas, which tend to adhere more tightly to the hypothalamus, and from which a small residue may quickly grow into a large cyst tumor. Therefore, we attempt total resection whenever possible by careful sharp dissection between the tumor and hypothalamus to avoid these postoperative complications.…”

Pediatric sellar teratoma – Case report and review of the literature

“…3 ,Video 1 ) [ 25 , 49 ]. In children, hypothalamic injury results in hyperphagia, obesity, neurocognitive deficits, and lower quality of life and should be avoided at all costs, especially before puberty, independent of the underlying diagnosis [ 50 – 52 ]. Other common complications after sellar/suprasellar surgery include diabetes insipidus (DI), endocrine disturbances or CSF leak [ 36 , 53 , 54 ].…”

Molecular biological features of cyst wall of adamantinomatous craniopharyngioma

“…On the whole, on the premise of minimizing hypothalamic damage, reasonable management and control of cyst wall and stem cells may be the most active treatment. Although treatment strategies for craniopharyngiomas are controversial, researchers all understand the limitations of current treatment and are optimistic about the prospect of molecular biology research 5 , 37 , 51 , 52 . Therefore, based on the existence of stem cells, if the cyst wall is left after surgery, we should actively seek more effective treatment methods, including radiotherapy.…”

“…Despite of deep location of the tumor and the adjacent vital structures such as hypothalamus, optic nerve, internal carotid artery, people are still undeterred by obstacles on the road to effective management of craniopharyngiomas. In the past, gross total resection (GTR) for ACP was seen as the primary treatment albeit with high rates of serious complications such as optic and endocrinological injury, while with the increasing of the understanding of craniopharyngioma, the improved treatment such as the Subtotal resection (STR) + radiotherapy can also have a sound outcome similar as GTR 4 , 5 .…”