• Chuan Zhao, Zhongqing Zhou, Yongli Zhang, Xueling Qi, Xianlong Wang, Da Lin, Hongxing Liu, Ye Wang, Chaoqun Weng, Jintao Chen, Wenxin Hu, Lianming Liao, and Zhixiong Lin.
• Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
• World Neurosurg. 2022 Sep 1; 165: e148e158e148-e158.

ObjectivePapillary craniopharyngioma (PCP) was previously believed to occur only in adults. Sporadic pediatric PCP (PPCP) confirmed by detection of BRAF V600E mutation has been reported since 2018, but is often misdiagnosed before being diagnosed definitively. We aimed to evaluate PPCP characteristics and propose diagnostic criteria for prompt diagnosis, seeking to reduce patient morbidity and mortality and reduce costs linked to misdiagnosis.MethodsThis study included 5 patients with PPCPs whose data were retrieved retrospectively from among 1032 patients with craniopharyngiomas admitted to Sanbo Brain Hospital Management Group from March 2017 to May 2021. Patients' demographics, clinical presentation, tumor imaging characteristics, histopathologic results, surgical approaches, and postoperative outcomes were analyzed.ResultsPPCP was misdiagnosed intraoperatively as sellar abscess (n = 4) or Rathke cleft cyst (n = 1). Preoperative magnetic resonance imaging showed that all tumors were under the saddle diaphragm, and the cyst wall was enhanced (n = 5). Computed tomography scans showed scattered high-density signs (n = 4). No recurrence was noted after complete resection. Postoperative hypothalamic dysfunction was mild. BRAF V600E mutation was confirmed in all cases by sequencing and immunohistochemistry. Immunohistochemistry showed granulation and inflammation and MPO, CD3, CD20, CD38, CD68, and CD163 were positively expressed.ConclusionsMisdiagnosis of PPCP is responsible for failed surgical treatment. We propose that prompt diagnosis of PPCP can be achieved if preoperative magnetic resonance images show the tumor under saddle diaphragm with tumor wall enhancement and computed tomography scans show high-density signs scattered in the tumor, which leads to safe, effective tumor resection. Our proposed diagnosis and treatment strategy for PPCP reduces morbidity and mortality.Copyright © 2022 Elsevier Inc. All rights reserved.

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