A modern series of subdiaphragmatic craniopharyngiomas

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OBJECTIVE

The endoscopic endonasal approach has been proposed as a primary surgical strategy for select craniopharyngiomas. However, those tumors that arise from the sella have not been classified with the other craniopharyngioma subtypes in terms of surgical nuances, intraoperative findings, and postoperative outcomes. The authors describe their experience with a select subtype of craniopharyngioma arising within the sella subjacent to the diaphragma sellae and refer to these tumors as type 0.

METHODS

After obtaining IRB approval, three institutions retrospectively reviewed their data collected from 2005 to 2017. Patients eligible for inclusion in the study were those who had tumors that originated from the sella inferior to the diaphragma sellae. Demographic, clinical, radiological, surgical, and follow-up data were examined and analyzed.

RESULTS

Twenty-eight patients (average age 19.3 years, range 3–60 years) were included in this study. Sixteen patients (57%) were younger than 18 years of age. All patients had characteristic imaging features of an expanded sella. Seventy-five percent of the patients presented with some form of visual field deficit (89% had radiographic optic apparatus compression) and 39% with hypopituitarism. The average maximal tumor diameter in the axial, coronal, or sagittal plane was 3.1 cm. Gross-total resection was achieved in 82% of the patients. Twenty-one percent of patients experienced an iatrogenic complication, and there were only two cases (7%) of postoperative cerebrospinal fluid (CSF) leakage. Only two patients (7%) required the use of a nasoseptal flap as part of their original reconstruction. Pathology was uniformly the adamantinomatous subtype. Postoperative objective visual outcomes were improved in 71% of the patients with visual symptoms or visual field deficits on presentation and stable in 24%. Mean follow-up was 45.1 months (range 3–120 months) with an 18% recurrence rate at a mean of 44.4 months (range 10–84 months). One patient was lost to follow-up. Thirty-six percent of patients received postoperative radiation to treat recurrence or residual tumor. Endocrine data are also presented.

CONCLUSIONS

Craniopharyngiomas that originate within the sella below the diaphragma sellae are a select subtype characterized by 1) an enlarged sella, 2) an intact diaphragma sellae at surgery, and 3) an adamantinomatous pathology. These tumors can be treated transnasally without the absolute need for neurovascular flap reconstruction, as there is a low risk of CSF leakage.

ABBREVIATIONS DI = diabetes insipidus; EEA = endoscopic endonasal approach.

Article Information

Correspondence Daniel Prevedello: Ohio State University, Wexner Medical Center, Columbus, OH. daniel.prevedello@osumc.edu.

INCLUDE WHEN CITING Published online October 26, 2018; DOI: 10.3171/2018.4.JNS172330.

Disclosures Dr. Prevedello has been a consultant for Stryker, Medtronic, and Codman; holds a patent with KLS-Martin; has received honorarium from Leica Microsystems; and has received clinical or research support from Storz for the study described. Dr. Carrau is a consultant for Medtronic.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Box-and-whisker plot of maximal tumor dimensions between patients with complications and those with no complications.

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    Box-and-whisker plot of maximal tumor dimensions between patients with residual tumor and those with no residual tumor.

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    Case 1. A: Preoperative sagittal contrast-enhanced MR image showing a sellar/suprasellar mass. B: Postoperative MR image showing possible intrasellar residual tumor. C: Tumor recurrence. D: Image obtained post-EEA for resection of recurrent tumor confined to the sella.

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    Case 2. A: Preoperative postcontrast coronal MR image showing tumor expanding the sella and extending into the sphenoid sinus. B: Postoperative enhancing coronal MR image revealing a recurrence in the right parasellar compartment. C: Image obtained after a second EEA for resection of recurrent disease, showing no evidence of residual tumor. D: Coronal MR image obtained about 7 months after the second EEA, showing an asymptomatic recurrence.

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    Case 3. A: Postcontrast coronal T1-weighted MR image revealing a sellar mass with a significant suprasellar cyst, causing compression of the optic apparatus as well as the third ventricle. B: Coronal T2-weighted MR image recapitulating these findings and emphasizing the cystic component. C: Sagittal MR image revealing tumor invasion into the sphenoid sinus inferiorly. D: Sagittal CT angiogram does not reveal compression of the anterior cerebral artery complex.

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    Case 3. Intraoperative image (A) showing the mammillary body (MB), pituitary stalk (PS), and optic chiasm (OC). Asterisk indicates an intact third ventricle floor. Postoperative sagittal (B) and coronal (C) postcontrast MR images show gross-total resection. Figure is available in color online only.

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