Prevalence of Neurobehavioral, Social, and Emotional Dysfunction in Patients Treated for Childhood Craniopharyngioma: A Systematic Literature Review

Zada, Gabriel; Kintz, Natalie; Marquez, Mario Pulido; Amezcua, Lilyana

doi:10.1371/journal.pone.0076562

Cited by 81 publications

(69 citation statements)

References 40 publications

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“…Several studies have shown dramatic reductions in memory, executive functions, and QOL in pediatric patients with craniopharyngiomas, particularly those with hypothalamic involvement, with as many as 57% showing neurobehavioral dysfunction. 46 Three prior studies have examined QOL in adult patients. The more recent study included 29 cured adults with 19 years of follow-up and compared results of 4 health-related questionnaires with those administered to 142 matched controls.…”

Section: Discussionmentioning

confidence: 99%

“…At time of presentation, and certainly after treatment, craniopharyngiomas can be associated with visual field defects, endocrinopathies, hypothalamic syndromes, increased cardiovascular risk, and neurocognitive sequelae. 7,9,33,37,46 Although considered benign tumors, their location can impact critical structures such as the optic nerves and chiasm, hypoabbreviatioNs ASBQ = Anterior Skull Base Quality of Life; BMI = body mass index; GTR = gross-total resection; QOL = quality of life; obJect Craniopharyngiomas are benign parasellar tumors for which surgical removal, although potentially curative, often leads to morbidity with resulting decreases in quality of life (QOL). The endonasal endoscopic approach is a minimal-access technique for removing these tumors and may reduce postoperative morbidity.…”

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Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas

Patel

Raza

McCoul³

et al. 2015

JNS

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T he management of craniopharyngiomas is fraught with challenges that not only hinder achievement of optimal oncological outcomes but potentially have adverse effects on the quality of life (QOL). At time of presentation, and certainly after treatment, craniopharyngiomas can be associated with visual field defects, endocrinopathies, hypothalamic syndromes, increased cardiovascular risk, and neurocognitive sequelae. 7,9,33,37,46 Although considered benign tumors, their location can impact critical structures such as the optic nerves and chiasm, hypoabbreviatioNs ASBQ = Anterior Skull Base Quality of Life; BMI = body mass index; GTR = gross-total resection; QOL = quality of life; obJect Craniopharyngiomas are benign parasellar tumors for which surgical removal, although potentially curative, often leads to morbidity with resulting decreases in quality of life (QOL). The endonasal endoscopic approach is a minimal-access technique for removing these tumors and may reduce postoperative morbidity. The QOL following this method for resection of craniopharyngiomas has not been documented. methods The authors reviewed a database of consecutive endonasal endoscopic surgeries done at Weill Cornell Medical College. Adult patients with histologically proven craniopharyngiomas were included who had completed either only postoperative (> 9 months) or both pre- and postoperative QOL forms, the Anterior Skull Base Quality of Life (ASBQ) questionnaire, and the 22-Item Sinonasal Outcome Test (SNOT-22). Rates of gross-total resection (GTR), complications, and visual and endocrine function were collected. Retrospective independence (Wen score) was also assigned. A contemporaneous group of patients undergoing endonasal endoscopic pituitary macroadenoma resection was used as a control. results This study included 33 procedures performed in 31 patients. The average postoperative ASBQ score was 3.35 and the SNOT-22 score was 19.6. Better QOL was associated with GTR and postoperative radiation. Worse QOL was associated with persistent visual defects, hypopituitarism, tumor recurrence, increase in body mass index, and worsening Wen score. In a subset of 10 patients, both pre- and postoperative (> 9 months) QOL scores were obtained. Both ASBQ and SNOT-22 scores showed stability and a trend toward improvement, from 2.93 ± 0.51 to 2.96 ± 0.47 (ASBQ) and . Compared with 62 patients undergoing endoscopic pituitary macroadenoma resection, patients with craniopharyngiomas had worse postoperative QOL on the ASBQ (3.35 vs 3.80; p = 0.023) and p = 0.12). coNclusioNs This report of validated site-specific QOL following endoscopic surgery for craniopharyngiomas shows an overall maintenance of postoperative compared with preoperative QOL. Better QOL could be seen in patients with GTR and radiation therapy, and worse QOL was found in patients with visual or endocrine deficits. Nevertheless, patients with craniopharyngiomas still had worse QOL than those undergoing similar surgery for pituitary macroadenomas, confirming the worse prognosis of craniop...

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas

Patel

Raza

McCoul³

et al. 2015

JNS

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“…Neurocognitive and neurobehavioral dysfunctions are still among the primary concerns for complications in survivors of childhood craniopharyngioma (31)(32)(33)(34). Previous studies have reported that post-therapeutic cognitive and behavioral outcomes were implicated by normal brain volume receiving a high radiation dose and various surgical factors such as CSF shunting, presence of ommaya reservoir, and extent of resection (32,33).…”

Section: Effect Of Surgical Defects and Implicationsmentioning

confidence: 99%

Effects of Surgery and Proton Therapy on Cerebral White Matter of Craniopharyngioma Patients

Hua

Ogg

et al. 2014

International Journal of Radiation Oncology*Biology*Physics

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Purpose-To determine the radiation dose-effect on the structural integrity of cerebral white matter in craniopharyngioma patients receiving surgery and proton therapy.Methods and Materials-Fifty-one patients (aged 2.1-19.3 years) with craniopharyngioma underwent surgery and proton therapy in a prospective therapeutic trial. Anatomical magnetic resonance images acquired after surgery but before proton therapy were inspected to identify white matter structures intersected by surgical corridors and catheter tracks. Longitudinal diffusion tensor imaging (DTI) was performed to measure microstructural integrity changes in cerebral white matter. Fractional anisotropy (FA) derived from DTI was statistically analyzed for 51 atlasbased white matter structures of the brain to determine radiation dose-effect. FA in surgeryaffected regions in the corpus callosum was compared to that in its intact counterpart to determine whether surgical defects affect radiation dose-effect.Results-Surgical defects were seen most frequently in the corpus callosum because of transcallosal resection of tumors and insertion of ventricular or cyst catheters. The longitudinal DTI data indicated reductions in FA 3 months after therapy, which was followed by a recovery in most white matter structures. A greater FA reduction was correlated with a higher radiation dose in 20 white matter structures, indicating a radiation dose-effect. The average FA in the surgery- Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. HHS Public Access Author Manuscript Author ManuscriptAuthor ManuscriptAuthor Manuscript affected regions before proton therapy was smaller (P =0.0001) than that in their non-surgery affected counterparts with more intensified subsequent reduction of FA (P=0.0083) after therapy, suggesting that surgery accentuated the radiation dose-effect.Conclusions-DTI data suggest that mild radiation dose-effects occur in patients with craniopharyngioma receiving surgery and proton therapy. Surgical defects present at the time of proton therapy appear to accentuate the radiation dose-effect longitudinally. This study supports consideration of pre-existing surgical defects and their locations in proton therapy planning and studies of treatment effect.

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“…1,44 The lesion is histologically benign but, due to its proximity to the critical neurovascular and hypothalamopituitary axis structures, it poses challenges to the treating physician. 1,61 Surgical or adjuvant treatment strategies in this eloquent location may have devastating effects, especially in the pediatric population in whom hypothalamic injury can result in the development of morbid obesity and cognitive decline. 21 Almost all pediatric craniopharyngiomas are of the adamantinomatous type with a CTNNB1 mutation, and they are generally calcified and thus not only more difficult to resect but also not responsive to medical therapy.…”

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Suprasellar and recurrent pediatric craniopharyngiomas: expanding indications for the extended endoscopic transsphenoidal approach

Alalade¹,

Ogando‐Rivas²,

Boatey³

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEThe expanded endonasal endoscopic transsphenoidal approach has become increasingly used for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, in recurrent and irradiated tumors, and in masses primarily located in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to application in older children with mostly cystic craniopharyngiomas centered in the sella. The authors present a series of consecutive pediatric patients in whom the endonasal endoscopic approach was used to remove craniopharyngiomas from patients of varied ages, regardless of the location of the tumor and previous treatments or surgeries, to ascertain if the traditional concerns about limitations of this approach are worth reevaluatingMETHODSEleven consecutive pediatric patients (age ≤ 18 years) underwent surgery via an endoscopic transsphenoidal approach at NewYork-Presbyterian/Weill Cornell Medical Center from 2007 to 2016. The authors recorded the location, consistency, and size of the lesion, assessed for hypothalamic invasion radiographically, calculated skull base measurements, and assessed parameters such as extent of resection, visual function, endocrinological function, weight gain, and return-to-school status.RESULTSThe average age at the time of surgery was 7.9 years (range 4–17 years) and the tumor sizes ranged from 1.3 to 41.7 cm3. Five cases were purely suprasellar, 5 had solid components, 4 were reoperations, and 5 had a conchal sphenoid aeration. Nevertheless, gross-total resection was achieved in 45% of the patients and 50% of those in whom it was the goal of surgery, without any correlation with the location, tumor consistency, or the age of the patient. Near-total resection, subtotal resection, or biopsy was performed intentionally in the remaining patients to avoid hypothalamic injury. Anterior pituitary dysfunction occurred in 81.8% of the patients, and 63.3% developed diabetes insipidus . Two patients (18%) had a greater than 9% increase in body mass index. Visual function was stable or improved in 73%. All children returned to an academic environment, with 10 of them in the grade appropriate for their age. There was a single case of each of the following: CSF leak, loss of vision unilaterally, and abscess.CONCLUSIONSThe endoscopic transsphenoidal approach is suitable for removing pediatric craniopharyngiomas even in young children with suprasellar tumors, conchal sphenoid sinus, recurrent tumors, and tumors with solid components. The extent of resection is dictated by intrinsic hypothalamic tumor invasiveness rather than the approach. The endoscopic transsphenoidal approach affords the ability to directly inspect the hypothalamus to determine invasion, which may help spare the patient from hypothalamic injury. Irrespective of approach, the rates of postoperative endocrinopathy remain high and the learning curve for the approach to a relatively rare tumor is steep.

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Prevalence of Neurobehavioral, Social, and Emotional Dysfunction in Patients Treated for Childhood Craniopharyngioma: A Systematic Literature Review

Cited by 81 publications

References 40 publications

Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas

Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas

Effects of Surgery and Proton Therapy on Cerebral White Matter of Craniopharyngioma Patients

Suprasellar and recurrent pediatric craniopharyngiomas: expanding indications for the extended endoscopic transsphenoidal approach

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