Mini-review: Childhood-onset Craniopharyngioma

2021 ◽  
Author(s):  
Anna Otte ◽  
Hermann L Müller
Keyword(s):  
Quality Of Life ◽  
Survival Rates ◽  
Residual Tumor ◽  
Tumor Location ◽  
Multidisciplinary Teams ◽  
Low Grade ◽  
Childhood Onset ◽  
Severe Impairment ◽  
Grade Malignancy

Abstract Craniopharyngiomas are rare embryonic malformational tumors of the sellar/parasellar region, classified by the WHO as tumors with low-grade malignancy (WHO I°). The childhood adamantinomatous subtype of craniopharyngioma is usually cystic with calcified areas. At the time of diagnosis, hypothalamic/pituitary deficits, visual disturbances and increased intracranial pressure are major symptoms. The treatment of choice in case of favorable tumor location (without hypothalamic involvement) is complete resection. It is important to ensure that optical and hypothalamic functionality are preserved. In case of unfavorable tumor location, i.e. with hypothalamic involvement, a hypothalamus-sparing surgical strategy with subsequent local irradiation of residual tumor is recommended. In the further course of the disease, recurrences and progression often occur. Nevertheless, overall survival rates are high at 92%. Severe impairment of quality of life and comorbidities such as metabolic syndrome, hypothalamic obesity and neurological consequences can be observed in patients with disease- and/or treatment-related lesions of hypothalamic structures. Childhood-onset craniopharyngioma frequently manifests as a chronic disease so that patients require lifelong, continuous care by experienced multidisciplinary teams to manage clinical and quality of life consequences.For this review, a search for original articles and reviews published between 1986 and 2020 was performed in Pubmed, Science Citation Index Expanded, EMBASE and Scopus. The search terms used were “craniopharyngioma, hypothalamus, pituitary obesity, irradiation, neurosurgery”.

scholarly journals Craniopharyngioma

2014 ◽  
Vol 35 (3) ◽  
pp. 513-543 ◽  
Author(s):  
Hermann L. Müller
Keyword(s):  
Quality Of Life ◽  
Histological Grade ◽  
Survival Rates ◽  
Age Distribution ◽  
Clinical Manifestations ◽  
Incidence Rates ◽  
Multidisciplinary Teams ◽  
Childhood Onset ◽  
Cognitive Problems

Abstract This report is a review of findings on the diagnosis, treatment, clinical course, and prognosis of craniopharyngioma patients. Craniopharyngiomas are rare, partly cystic and calcified embryonic malformations of the sellar/parasellar region with low histological grade (WHO I°). A bimodal age distribution has been shown, with peak incidence rates in childhood-onset at 5–14 years and adult-onset craniopharyngioma at 50–74 years. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve optical and hypothalamic functions. In patients with unfavorable tumor localization (ie, hypothalamic involvement), recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Although overall survival rates are high (92%), recurrences and progressions are frequent. Irradiation has proven effective in reducing recurrences and progression, and timing of postsurgical irradiation in childhood-onset cases is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious quality of life-compromising sequelae such as hypothalamic obesity, psychopathological symptoms, and/or cognitive problems. It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and quality of life consequences by experienced multidisciplinary teams.

scholarly journals Quality of life in patients with narcolepsy: a WHOQOL-bref study

2008 ◽  
Vol 66 (2a) ◽  
pp. 163-167 ◽  
Author(s):  
Heloísa Rovere ◽  
Sueli Rossini ◽  
Rubens Reimão
Keyword(s):  
Quality Of Life ◽  
Patient Group ◽  
World Health ◽  
Control Group ◽  
World Health Organization Quality ◽  
Severe Impairment ◽  
Perception Of Quality ◽  
Social Domains ◽  
Health Organization

OBJECTIVE: To evaluate the perception of Quality of Life (QL) in Brazilian patients with narcolepsy. METHOD: 40 adult patients aged between 20 and 72 years (mean=41.55; SD=14.50); (28 F; 12M), with the diagnosis of chronic narcolepsy were followed up at the outpatient clinic (Patient Group). The Control Group was composed of 40 adults. The instrument utilized was the World Health Organization Quality of Life (WHOQOL-BREF). RESULTS: The two groups were homogeneous and no difference was found with regards to age, sex, and demographic characteristics. The perception of QL in physical, psychological and social domains showed lower scores in those patients with narcolepsy than in the control group (p<0.05). Concerning physical domain, all the aspects evaluated were significantly impaired, in patient group, including sleep satisfaction (p<0.001); energy for daily activities (p=0.039); capacity to perform activities (p=0.001); and capacity to work (p=0.001). CONCLUSION: The perception of QL showed severe impairment in patients with narcolepsy for physical, psychological and social domains.

scholarly journals QOL-36. USE OF CANNABINOIDS IN THE PEDIATRIC CENTRAL NERVOUS SYSTEM TUMOR POPULATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii438-iii438
Author(s):  
Kathleen Dorris ◽  
Jessica Channell ◽  
Ashley Mettetal ◽  
Molly Hemenway ◽  
Natalie Briones ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Central Nervous System ◽  
Nervous System ◽  
Cell Activity ◽  
Cns Tumors ◽  
Low Grade ◽  
Tumor Type ◽  
Central Nervous System Tumor ◽  
The Impact

Abstract BACKGROUND Cannabinoids, including cannabidiol (CBD) and tetrahydrocannabinol (THC), are a class of compounds found in marijuana. Numerous studies in adults have examined cannabinoid use in management of cancer-related symptoms such as nausea, anorexia, and pain. Less is known about the use in the pediatric oncology population. METHODS A prospective observational study has been ongoing since 2016 at Children’s Hospital Colorado to evaluate cannabinoids’ impact using PedsQL™ modules on quality of life of pediatric patients with central nervous system (CNS) tumors who are 2–18 years old. Laboratory assessments of T-cell activity and pharmacokinetics of CBD, THC and associated metabolites are in process. Diaries with exploratory information on cannabinoid use patterns are being collected. RESULTS Thirty-three patients (14:19; male:female) have been enrolled with a median age of 6.4 years (range, 2.9–17.7 years). The most common tumor type in enrolled patients is embryonal tumors (13/33; 39%). Nine (27%) patients have low-grade glial/glioneuronal tumors, and eight (24%) had high-grade/diffuse midline gliomas. The remaining patients had ependymoma or craniopharyngioma. The median time on cannabinoids is 9 months. Most (n=20) patients have used oral products with CBD and THC. One patient continues on cannabinoid therapy in follow up. Preliminary immune function analyses identified impaired neutrophil superoxide anion production and chemotaxis in patients taking cannabinoids at early time points on therapy. CONCLUSIONS Families of children with various CNS tumors are pursuing cannabinoid therapy for both antitumor and supportive care purposes. Analysis of the impact of cannabinoids on patients’ quality of life is ongoing.

scholarly journals Challenges and Perspectives on Breast Cancer Survivorship: The Journey Continues

2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 96-96
Author(s):  
Victoria Raveis ◽  
Simona Kwon
Keyword(s):  
Breast Cancer ◽  
Quality Of Life ◽  
Cancer Survivor ◽  
Breast Cancer Survivors ◽  
Cancer Survival ◽  
Survival Rates ◽  
Extended Period ◽  
Health Issues ◽  
Age Related

Abstract Women have a 1-in-8 lifetime risk of breast cancer. Earlier diagnosis and treatment advances have improved 15- and 20-year survival rates. Increased survival can mean coping with the effects of cancer and its treatment over an extended period of time, while experiencing age-related changes in functioning and the emergence of other health issues. To explore breast cancer survivors’ perspectives on their issues and concerns across the life-course, focus groups were conducted with a culturally diverse sample (N=18) of survivors (72% white, 28% Black, 11% Hispanic). Participants were 44-82 years old. Most, 83% were 50 and older, 56% were 60 and older. The majority (83%) were diagnosed in their 40’s and 50’s. Two were diagnosed in their early 30’s and one at age 68. Participants reaffirmed the necessity, as a breast cancer survivor, of being a life-long health advocate on their own behalf, and the importance of being self-informed. As one woman commented: “Knowledge is power”. Survivors shared that their emergent health issues were complicated by their cancer history, and, that, as a cancer survivor, “I never stop worrying”. A widespread concern was not knowing if the health issues and co-morbidities they experienced (such as joint pain, neuropathy, tendinitis, heart disease), were age-related, a consequence of their cancer, or a late treatment effect. An overriding sentiment expressed was that clinicians have not recognized the importance of quality of life in cancer survival. As a survivor succinctly stated: “We are living longer, but we need to live long with quality of life.”

scholarly journals Endoscopic Endonasal Transethmoidal-Transsphenoidal Approach to a Cavernous Sinus Chondrosarcoma

2021 ◽  
Author(s):  
Gianluca Agresta ◽  
Alberto Campione ◽  
Fabio Pozzi ◽  
Pierlorenzo Veiceschi ◽  
Martina Venturini ◽  
...  
Keyword(s):  
Skull Base ◽  
Cavernous Sinus ◽  
Conformal Radiotherapy ◽  
Residual Tumor ◽  
Complete Recovery ◽  
Tumor Location ◽  
Transsphenoidal Approach ◽  
Low Grade ◽  
Endoscopic Endonasal ◽  
Skull Base Reconstruction

Abstract Objective We illustrate a cavernous sinus chondrosarcoma treated with an endoscopic endonasal transethmoidal-transsphenoidal approach. Design Case report of a 15-year-old girl with diplopia and esotropia due to complete abducens palsy. Preoperative images showed a right cavernous sinus lesion with multiple enhanced septa and intralesional calcified spots (Fig. 1). Considering tumor location and the lateral dislocation of the carotid artery, an endoscopic endonasal approach was performed to relieve symptoms and to optimize the target geometry for adjuvant conformal radiotherapy. Setting The study was conducted at University of Insubria, Department of Neurosurgery, Varese, Italy. Participants Skull base team was participated in the study. Main Outcome Measures A transethmoidal-transsphenoidal approach was performed by using a four-hand technique. We used a route lateral to medial turbinate to access ethmoid and the sphenoid sinus. During the sphenoid phase, we exposed the medial wall of the cavernous sinus (Fig. 2) and the lesion was then removed using curette. Skull base reconstruction was performed with fibrin glue and nasoseptal flap. Results No complications occurred after surgery, and the patient experienced a complete recovery of symptoms. A postoperative magnetic resonance imaging showed a small residual tumor inside the cavernous sinus (Fig. 1). After percutaneous proton-bean therapy, patient experienced only temporary low-grade toxicity with local control within 2 years after treatment completion. Conclusion Endoscopic endonasal extended approach is a safe and well-tolerated procedure that is indicated in selected cases (intracavernous tumors, soft tumors not infiltrating the vessels and/or the nerves). A tailored approach according to tumor extension is crucial for the best access to the compartments involved.The link to the video can be found at: https://youtu.be/TsqXjqpuOws.

scholarly journals A Dutch phase III randomized multicenter trial: whole brain radiotherapy vs. stereotactic radiotherapy for 4-10 brain metastases

2021 ◽  
Author(s):  
Dianne Hartgerink ◽  
Anna Bruynzeel ◽  
Danielle Eekers ◽  
Ans Swinnen ◽  
Coen Hurkmans ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Side Effects ◽  
Brain Metastases ◽  
Survival Rates ◽  
Whole Brain Radiotherapy ◽  
Phase Iii ◽  
Whole Brain ◽  
Brain Radiotherapy ◽  
One Year

Abstract Background The clinical value of whole brain radiotherapy (WBRT) for brain metastases (BM) is a matter of debate due to the significant side effects involved. Stereotactic radiosurgery (SRS) is an attractive alternative treatment option that may avoid these side effects and improve local tumor control. We initiated a randomized trial (NCT02353000) to investigate whether quality of life is better preserved after SRS compared with WBRT in patients with multiple brain metastases. Methods Patients with 4 to 10 BM were randomized between the standard arm WBRT (total dose 20 Gy in 5 fractions) or SRS (single fraction or 3 fractions). The primary endpoint was the difference in quality of life (QOL) at three months post-treatment. Results The study was prematurely closed due to poor accrual. A total of 29 patients (13%) were randomized, of which 15 patients have been treated with SRS and 14 patients with WBRT. The median number of lesions were 6 (range, 4-9) and the median total treatment volume was 13.0 cc 3 (range, 1.8-25.9 cc 3). QOL at three months decreased in the SRS group by 0.1 (SD=0.2), compared to 0.2 (SD=0.2) in the WBRT group (p=0.23). The actuarial one-year survival rates were 57% (SRS) and 31% (WBRT) (p=0.52). The actuarial one-year brain salvage-free survival rates were 50% (SRS) and 78% (WBRT) (p=0.22). Conclusion In patients with 4 to 10 BM, SRS alone resulted in one-year survival for 57% of patients while maintaining quality of life. Due to the premature closure of the trial, no statistically significant differences could be determined.

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