Adamantinomatous craniopharyngioma: evolution in the management

Abstract

Background: In spite of the continuous progresses in pediatric neurosurgery, adamantinomatous craniopharyngioma (AC) remains a challenging tumor due to its proximity to optic pathways, pituitary gland, hypothalamus, and Willis' circle, which can result in significant endocrine, cognitive, and neurological morbidity after treatment with subsequent impact on the patient's quality of life (QoL). The relevance that QoL has today explains the changes in the management of AC observed over the time. The goal of the present article is to provide a historical background, to show the milestones in the changes of the AC treatment, and to analyze the current main options to manage such a challenging tumor.

Material and methods: The pertinent literature has been reviewed. Moreover, a comparison between the past and recent personal series is reported.

Results: Three main eras have been identified. The first (named Cushing era) was characterized by the need to realize a harmless surgery and to define the best way to approach AC; the second (microscope era) was characterized by a tremendous technical and technological development, with remarkable results in term of safe tumor resection and control but relatively poor QoL outcomes; and the third one (current period) is characterized by an increasing integration between surgery and adjuvant treatments, with relatively minor tumor control but significant improvement of QoL (comparable overall survival). The authors' experience reflects these changes. Two groups of children were compared: 52 cases (mean follow-up: 17.5 years) belong to the historical series (group 1, 1985-2003, aggressive surgical management) and 41 (mean follow-up: 8.5 years) to the current one (Group 2, 2004-2021, integrated management). No significant differences between the two groups were detected about recurrence rate, surgical mortality, and overall survival. However, Group 2 showed significant lower rates of postoperative panhypopituitarism, obesity, and visual deterioration.

Conclusions: Radical surgery allows for a good AC control with a low rate of recurrence but high risk of permanent morbidity. Despite the greater number of recurrences and surgeries, the more conservative policy, based on a combination of treatments, seems to provide the same tumor control with a better QoL. The advances in trans-nasal and trans-ventricular endoscopy, in proton therapy and in the management of the AC cyst are the main factors that allowed such an improvement.

Keywords: Adamantinomatous craniopharyngioma; Endoscopic endonasal transphenoidal surgery; Interferon alpha; Intracystic therapy; Management; Proton therapy; Quality of life.

Fig. 1

Giant AC in a 11-year-old boy. Note the large and enhancing cystic components in the T1 sequences after gadolinium administration (A, B, C), which extend into the anterior, middle, and posterior cranial fossa. Also a biventricular hydrocephalus is evident (A–E). The cyst fluid is hyperintense compared to CSF in FLAIR sequence (D). Note that the solid component is a minor part of the tumor (E, arrow) but involves the region of the Willis’ circle (F, asterisk)

Fig. 2

Pre-treatment T1 sagittal (A), axial (B), and coronal MRI (C) of AC in a 5-year-old girl. Note the relatively small, solid, sella portion, and the large suprasellar/third ventricle cystic component. The same sequences performed 2 years later (D, E, F), at the end of 3 cycles of IA, show a quite good control of the disease, with shrinkage of the cyst. The cyst wall remains thick and present a “rigid” appearance

Fig. 3

Intraoperative view of the same case in Fig. 2 (the child was operated when she was 8 years old, 3 years after the IA treatment, because of initial tumor-regrowth). In spite of the subtraction of the cystic fluid (A, B, C), the tumor capsule remains somewhat fibrotic, thicker, and more difficult do detach from the surrounding structures, and it does not deflate after the cyst fluid removal (D, E, F). This makes the tumor excision more complicated (G, H, I)

Fig. 4

T1 MRI before (A) and after gadolinium administration (B) showing the presence of AC in a 7-year-old girl; C, D the same MRI sequences after navigation-guided placement of intracystic catheter and aspiration of motor-oil fluid, which allowed a significant reduction of the cyst with decompression of the surrounding structures; E, F CT scan performed at the last follow-up (4 years later and after 3 cycles of IA) showing the effacement of the cystic component with evidence of a small, calcified intrasellar, tumor solid portion