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Diagnosis and Management of Pediatric Papillary Craniopharyngiomas.
Zhao, Chuan; Zhou, Zhongqing; Zhang, Yongli; Qi, Xueling; Wang, Xianlong; Lin, Da; Liu, Hongxing; Wang, Ye; Weng, Chaoqun; Chen, Jintao; Hu, Wenxin; Liao, Lianming; Lin, Zhixiong.
Affiliation
  • Zhao C; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Zhou Z; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Zhang Y; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Qi X; Department of Pathology, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Wang X; Department of Bioinformatics, School of Medical Technology and Engineering, Key Laboratory of Ministry of Education for Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian, China.
  • Lin D; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China; Department of Neurosurgery, Beijing Luhe Hospital, Capital Medical University, Beijing, China.
  • Liu H; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China; Department of Neurosurgery, Ditan Hospital, Capital Medical University, Beijing, China.
  • Wang Y; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China; Department of Neurosurgery, the Second Affiliated Hospital, Xiamen Medical College, Xiamen, Fujian, China.
  • Weng C; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Chen J; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Hu W; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
  • Liao L; Center of Laboratory Medicine, Fujian Medical University Union Hospital, Fuzhou, Fujian, China.
  • Lin Z; Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China. Electronic address: linzx@ccmu.edu.cn.
World Neurosurg ; 165: e148-e158, 2022 09.
Article in En | MEDLINE | ID: mdl-35660482
OBJECTIVE: Papillary 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. METHODS: This 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. RESULTS: PPCP 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. CONCLUSIONS: Misdiagnosis 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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Pituitary Neoplasms / Craniopharyngioma / Central Nervous System Cysts Type of study: Diagnostic_studies / Observational_studies Limits: Adult / Child / Humans Language: En Journal: World Neurosurg Journal subject: NEUROCIRURGIA Year: 2022 Document type: Article Affiliation country: China Country of publication: United States

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Pituitary Neoplasms / Craniopharyngioma / Central Nervous System Cysts Type of study: Diagnostic_studies / Observational_studies Limits: Adult / Child / Humans Language: En Journal: World Neurosurg Journal subject: NEUROCIRURGIA Year: 2022 Document type: Article Affiliation country: China Country of publication: United States