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

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          Abstract

          OBJECTIVE

          The 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 reevaluating

          METHODS

          Eleven 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.

          RESULTS

          The 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 cm 3. 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.

          CONCLUSIONS

          The 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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          Most cited references 55

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          The descriptive epidemiology of craniopharyngioma.

          In this report the authors describe the epidemiology of craniopharyngioma. The incidence of craniopharyngioma in the United States was estimated from two population-based cancer registries that include brain tumors of benign and borderline malignancy: the Central Brain Tumor Registry of the United States (CBTRUS) and the Los Angeles county Cancer Surveillance Program. Information on additional pediatric tumors was available from the Greater Delaware Valley Pediatric Tumor Registry (GDVPTR). The overall incidence of craniopharyngioma was 0.13 per 100,000 person years and did not vary by gender or race. A bimodal distribution by age was noted with peak incidence rates in children (aged 5-14 years) and among older adults (aged 65-74 years in CBTRUS and 50-74 years in Los Angeles county). Survival information was available from GDVPTR and the National Cancer Data Base (NCDB), a hospital-based reporting system. In the NCDB, the 5-year survival rate was 80% and decreased with older age at diagnosis. Survival is higher among children and has improved in recent years. Craniopharyngioma is a rare brain tumor of uncertain behavior that occurs at a rate of 1.3 per million person years. Approximately 338 cases of this disease are expected to occur annually in the United States, with 96 occurring in children from 0 to 14 years of age.
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            Craniopharyngioma in adults and children: a study of 122 surgical cases.

            This work is devoted to a 25-year retrospective study of 122 cases of craniopharyngiomas in adults and children treated and followed by the same neurosurgeon (R.V.E.). In this homogeneous series, the aim was total microsurgical removal of the tumor, without postoperative radiotherapy. The operation was performed via a frontopterional approach in 112 cases and a transsphenoidal approach in 10 cases. The tumor removal was considered total in 59%, subtotal in 29%, and partial in 12%. The surgical mortality rate was 2.5%. Even when tumor removal was not complete, radiotherapy was not systematically administered; it was reserved for cases of recurrence. The authors have studied clinical signs, operative characteristics, and ophthalmological, endocrinological, and functional outcomes, as well as recurrence risk and long-term patient survival. The mean follow-up period was 7 years. The functional results in these patients were excellent in 85%, good in 9%, fair in 5% (usually because of ophthalmological sequelae), and poor in 1%. Tumors recurred in 29 patients, but the salvage treatment, by operation or radiotherapy, was successful in 83%. The actuarial patient survival rate was 92% after 5 years and 85% after 10 years. These results compared favorably with the data reported in the literature, suggesting that radical surgery of craniopharyngiomas allows good outcome in terms of survival, full recovery, and quality of life for both adults and children.
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              Endoscopic, endonasal extended transsphenoidal, transplanum transtuberculum approach for resection of suprasellar lesions.

              The extended transsphenoidal approach is a less invasive method for removing purely suprasellar lesions compared with traditional transcranial approaches. Most advocates have used a sublabial incision and a microscope and have reported a significant risk of cerebrospinal fluid (CSF) leakage. The authors report on a series of purely endoscopic endonasal surgeries for resection of suprasellar supradiaphragmatic lesions above a normal-sized sella turcica with a low risk of CSF leakage. A purely endoscopic endonasal approach was used to remove suprasellar lesions in a series of 10 patients. Five lesions were prechiasmal (three tuberculum sellae and two planum sphenoidale meningiomas) and five were post-chiasmal (four craniopharyngiomas and one Rathke cleft cyst). The floor of the planum sphenoidale and the sella turcica was reconstructed using a multilayer closure with autologous and synthetic materials. Spinal drainage was performed in only five cases. Complete resection of the lesions was achieved in all but one patient. The pituitary stalk was preserved in all but one patient, whose stalk was invaded by a craniopharyngioma and who had preoperative diabetes insipidus (DI). Vision improved postoperatively in all patients with preoperative impairment. Six patients had temporary DI; in five, the DI became permanent. Four patients with craniopharyngiomas required cortisone and thyroid replacement. After a mean follow up of 10 months, there was only one transient CSF leak when a lumbar drain was clamped prematurely on postoperative Day 5. A purely endoscopic endonasal approach to suprasellar supradiaphragmatic lesions is a feasible minimally invasive alternative to craniotomy. With a multilayer closure, the risk of CSF leakage is low and lumbar drainage can be avoided. A larger series will be required to validate this approach.
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                Author and article information

                Journal
                Journal of Neurosurgery: Pediatrics
                Journal of Neurosurgery Publishing Group (JNSPG)
                1933-0707
                1933-0715
                January 2018
                January 2018
                : 72-80
                Article
                10.3171/2017.7.PEDS17295
                © 2018

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