Phase II trial of conformal radiation therapy for pediatric patients with craniopharyngioma and correlation of surgical factors and radiation dosimetry with change in cognitive function

Merchant, Thomas E.; Kiehna, Erin N.; Kun, Larry E.; Mulhern, Raymond K.; Li, Chenghong; Xu, Xiaoping; Boop, Frederick A.; Sanford, Robert A.

doi:10.3171/ped.2006.104.2.5

Cited by 106 publications

(107 citation statements)

References 18 publications

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“…Since our LTG was eventually associated with higher rate of RT (55 vs. 27 %, respectively; RR=2, 95 % CI 0.775-5.154), one can suggest to treat all large CP with STR+RT upfront. However, the issue of long-term toxicity caused by radiation is known to be critical in the pediatric population [26][27][28]. Furthermore, there are currently no level I or level II evidence studies comparing GTR to subtotal resection with or without radiation (regardless of tumor's size), so no strict recommendations, choosing one treatment modality over another, can be made.…”

Section: Adjuvant Radiation Therapymentioning

confidence: 97%

Giant craniopharyngiomas in children: short- and long-term implications

2015

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Tumor's size has significant impact on the management of CP in children. It affects both short-term factors (initial symptoms, urgency of surgical resection, extent of resection, and perioperative morbidity) as well as long-term parameters (PFS, rate of adjuvant treatments/recurrent surgeries, and metabolic/endocrinal/ophthalmological and functional outcomes). We think that a proper, world-wide accepted definition of what is "large," "giant," or even "monstrous" CP should be established. This will enable carrying multi-institutional studies on a larger group of patients, allowing further determining the importance of tumor's size in the management and outcome of craniopharyngiomas in children.

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Section: Adjuvant Radiation Therapymentioning

confidence: 97%

Giant craniopharyngiomas in children: short- and long-term implications

2015

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“…Therefore, the case encourages discussion on the most appropriate adjuvant therapy for young children with papillary craniopharyngiomas who have significant post-operative tumor residuum or progression. While stereotactic radiotherapy remains the recognized standard, concerns persist regarding neurocognitive, vascular, metabolic, and endocrinological sequelae, even with state-ofthe-art proton beam radiotherapy [14,20,21].…”

Section: Discussionmentioning

confidence: 99%

Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature

et al. 2018

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Introduction Craniopharyngiomas are one of the most frequently diagnosed hypothalamo-pituitary tumors in childhood. The adamantinomatous histological subtype accounts for most pediatric cases, while the papillary variant is almost exclusively diagnosed in adults. Here, we report a case of papillary craniopharyngioma in a very young child, confirmed by molecular tissue analysis. Case report A 4-year-old girl was being investigated for symptomatic central hypothyroidism. Brain MR imaging revealed a large solid/cystic suprasellar mass, splaying the optic chiasm and measuring 3 × 1.9 × 2.3 cm. The patient underwent a transsphenoidal near total resection of the lesion, which was encased within a tumor capsule. Post-operatively, the patient developed transient diabetes insipidus but otherwise recovered well. The pathology of the lesion was consistent with a papillary craniopharyngioma with regions of stratified squamous epithelium accompanied by superficial goblet cells and ciliated cells. Subsequent next-generation sequencing analysis of the lesion confirmed the presence of a BRAF V600E mutation (BRAFc.1799T>A p. (Val600Glu). To date, she remains free from progression 1 year following surgery. Conclusion This is the youngest case published to date of papillary craniopharyngioma with a confirmed BRAF V600E mutation. The case encourages discussion about the most appropriate adjuvant therapy for tumor progression in such cases, given the risks of radiotherapy to the developing brain and the increasing availability of oral BRAF inhibitor therapy.

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“…Today, fractionated irradiation with high-precision techniques using rigid head fixation is increasingly used [57,58]. By reducing the safety margins and sparing functional tissue, a reduction of inadvertent side-effects such as visual impairment, pituitary deficiency, impaired cognitive function or development of secondary malignancies can be anticipated [59,60].…”

Section: External Irradiationmentioning

confidence: 99%

Craniopharyngioma surgery

Honegger

Tatagiba

2008

Pituitary

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Ideal surgical treatment of craniopharyngiomas remains a major challenge for neurosurgeons. Craniopharyngiomas grow in the deep-seated hypothalamic area that is paramount for vegetative, emotional and endocrine function, and for maintaining worthwhile life. The benign histological nature of craniopharyngiomas belies their biological behavior and the propensity to recur is a major threat. Surgical treatment has to weigh the risk of hypothalamic damage against the risk of tumor recurrence or progression. Both aggressive surgery and conservative minor surgery followed by radiotherapy has been proclaimed by the proponents of different schools. During the past decade, the pendulum has swung back to surgery with the attempt at radical removal. Refined neurosurgical techniques and innovative approaches yielded improved surgical results. The contemporary neurosurgical strategy of treating craniopharyngiomas with early and late outcome data is presented. Neurosurgical therapy is only beneficial in the context of an interdisciplinary treatment concept as discussed here.

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Phase II trial of conformal radiation therapy for pediatric patients with craniopharyngioma and correlation of surgical factors and radiation dosimetry with change in cognitive function

Cited by 106 publications

References 18 publications

Giant craniopharyngiomas in children: short- and long-term implications

Giant craniopharyngiomas in children: short- and long-term implications

Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature

Craniopharyngioma surgery

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