“…Elevated levels of cytokines including IL-6, IL-8, CXCL1, and IL-10 have been documented in the cystic fluid of ACP (21). Notably, IL-6 appears to play a role in instigating an inflammatory response in adjacent non-tumor tissues (19,22). Furthermore, the expression patterns of these cytokines align with the activation of the inflammasome.…”
Section: Genetics and Inflammation In Acpmentioning
confidence: 99%
“…The limitations of the present literature on cyst management of ACP predominantly arise from the varying definitions of cystic recurrence in existing studies, making comparisons between different research endeavors challenging. Additionally, these clinical data primarily originate from patients treated in varying selective settings, often lacking extensive long-term follow-up (22), but these clinical experiences may open up a new potential avenue for the treatment of ACP.…”
Section: Stereotactic Cyst Drainagementioning
confidence: 99%
“…Given the potential reaggregation of cysts and the emergence of new clinical symptoms due to cystic occupation, an intracystic catheter and reservoir (such as a Rickham or Ommaya reservoir) are considered for implantation. In the majority of cases, it is feasible to establish a sustainable decompression regimen to address cyst progression (22,82). Mouss et al documented 52 patients with cystic craniopharyngioma, and with a minimum follow-up of 7 years, they were astounded to discover that 38 patients (73%) experienced no recurrence of cysts and necessitated no further treatment (81).…”
Section: Intracystic Catheter and Reservoir Systemmentioning
Adamantinomatous craniopharyngioma (ACP) presents a significant challenge to neurosurgeons despite its benign histology due to its aggressive behavior and unique growth patterns. This narrative review explores the evolving landscape of ACP treatments and their efficacy, highlighting the continuous development in therapeutic approaches in recent years. Traditionally, complete resection was the primary treatment for ACP, but surgical -related morbidity have led to a shift. The invasive nature of the finger-like protrusions in the histological structure results in a higher recurrence rate for ACP compared to papillary craniopharyngioma (PCP), even after complete macroscopic resection. Given this, combining subtotal resection with adjuvant radiotherapy has shown potential for achieving similar tumor control rates and potentially positive endocrine effects. Simultaneously, adjuvant treatments (such as radiotherapy, intracystic treatment, and catheter implantation) following limited surgery offer alternative approaches for sustained disease control while minimizing morbidity and alleviating clinical symptoms. Additionally, advances in understanding the molecular pathways of ACP have paved the way for targeted drugs, showing promise for therapy. There is a diversity of treatment models for ACP, and determining the optimal approach remains a subject of ongoing debate in the present context. In order to achieve a good-term quality of life (QOL), the main goal of the cyst disappearance or reduction of surgical treatment is still the main. Additionally, there should be a greater emphasis on personalized treatment at this particular stage and the consideration of ACP as a potentially chronic neurosurgical condition. This review navigates the evolving landscape of ACP therapies, fostering ongoing discussions in this complex field.
“…Elevated levels of cytokines including IL-6, IL-8, CXCL1, and IL-10 have been documented in the cystic fluid of ACP (21). Notably, IL-6 appears to play a role in instigating an inflammatory response in adjacent non-tumor tissues (19,22). Furthermore, the expression patterns of these cytokines align with the activation of the inflammasome.…”
Section: Genetics and Inflammation In Acpmentioning
confidence: 99%
“…The limitations of the present literature on cyst management of ACP predominantly arise from the varying definitions of cystic recurrence in existing studies, making comparisons between different research endeavors challenging. Additionally, these clinical data primarily originate from patients treated in varying selective settings, often lacking extensive long-term follow-up (22), but these clinical experiences may open up a new potential avenue for the treatment of ACP.…”
Section: Stereotactic Cyst Drainagementioning
confidence: 99%
“…Given the potential reaggregation of cysts and the emergence of new clinical symptoms due to cystic occupation, an intracystic catheter and reservoir (such as a Rickham or Ommaya reservoir) are considered for implantation. In the majority of cases, it is feasible to establish a sustainable decompression regimen to address cyst progression (22,82). Mouss et al documented 52 patients with cystic craniopharyngioma, and with a minimum follow-up of 7 years, they were astounded to discover that 38 patients (73%) experienced no recurrence of cysts and necessitated no further treatment (81).…”
Section: Intracystic Catheter and Reservoir Systemmentioning
Adamantinomatous craniopharyngioma (ACP) presents a significant challenge to neurosurgeons despite its benign histology due to its aggressive behavior and unique growth patterns. This narrative review explores the evolving landscape of ACP treatments and their efficacy, highlighting the continuous development in therapeutic approaches in recent years. Traditionally, complete resection was the primary treatment for ACP, but surgical -related morbidity have led to a shift. The invasive nature of the finger-like protrusions in the histological structure results in a higher recurrence rate for ACP compared to papillary craniopharyngioma (PCP), even after complete macroscopic resection. Given this, combining subtotal resection with adjuvant radiotherapy has shown potential for achieving similar tumor control rates and potentially positive endocrine effects. Simultaneously, adjuvant treatments (such as radiotherapy, intracystic treatment, and catheter implantation) following limited surgery offer alternative approaches for sustained disease control while minimizing morbidity and alleviating clinical symptoms. Additionally, advances in understanding the molecular pathways of ACP have paved the way for targeted drugs, showing promise for therapy. There is a diversity of treatment models for ACP, and determining the optimal approach remains a subject of ongoing debate in the present context. In order to achieve a good-term quality of life (QOL), the main goal of the cyst disappearance or reduction of surgical treatment is still the main. Additionally, there should be a greater emphasis on personalized treatment at this particular stage and the consideration of ACP as a potentially chronic neurosurgical condition. This review navigates the evolving landscape of ACP therapies, fostering ongoing discussions in this complex field.
“…1 ). In addition, AC is particularly prone to recurrence, even after an apparently gross total resection (GTR) [ 6 ]. Fig.…”
Section: Introductionmentioning
confidence: 99%
“…Afterwards, EETA, which was more and more diffused and technically improved, gained a relevant consent because of some obvious advantages (direct access to the tumor, no brain manipulation) [ 125 , 126 ]. At present, finally, it has been realized that these two approaches are both effective but should be performed in different population of children, alone (EETA in patients with mainly sellar/suprasellar midline AC while TMA in patients with large and laterally extending AC) or even in combination (huge AC) [ 6 , 89 ].…”
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.
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