Fang Wang1, Yanjiao He2, Chunde Li3, Yonggang Wang3, Liyong Zhong4. 1. Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. 2. Department of Neuropathology, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. 3. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. 4. Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. Electronic address: zhongliyong@126.com.
Abstract
BACKGROUND: Malignant craniopharyngioma is a rare clinical entity; most patients with this disease show transformation from an initially benign craniopharyngioma. The rare prevalence of the disease, nonspecific presenting symptoms, and imaging features that overlap with benign craniopharyngiomas make preoperative identification challenging. METHODS: In this study, we retrospectively evaluated the data of 7 patients diagnosed with malignant craniopharyngioma within a 5-year period in a single center. A thorough review of patient records from Beijing Tiantan Hospital, Capital Medical University, between 2013 and 2018 was performed. After identifying these 7 patients with malignant craniopharyngioma, we evaluated their demographic and clinical characteristics, including symptoms at presentation, imaging characteristics, treatment, and outcomes. RESULTS: Five patients showed transformation from benign craniopharyngiomas, and 2 had de novo malignancy. Two patients were children at diagnosis of malignancy. Patients' average age was 22 years. Headache was the most common presenting symptom, followed by visual field defects and adenohypophyseal dysfunction. Imaging characteristics showed that all patients had giant mass in the sellar, suprasellar, and/or parasellar regions that had invaded surrounding anatomic structures. Of the 5 patients who showed transformation from benign craniopharyngioma, 3 had undergone radiologic treatment before the diagnosis of malignant transformation. CONCLUSIONS: Malignant craniopharyngioma should not be overlooked in patients with a giant sellar mass, especially in young patients who have experienced multiple recurrences of craniopharyngioma and patients who received radiologic therapy.
BACKGROUND:Malignant craniopharyngioma is a rare clinical entity; most patients with this disease show transformation from an initially benign craniopharyngioma. The rare prevalence of the disease, nonspecific presenting symptoms, and imaging features that overlap with benign craniopharyngiomas make preoperative identification challenging. METHODS: In this study, we retrospectively evaluated the data of 7 patients diagnosed with malignant craniopharyngioma within a 5-year period in a single center. A thorough review of patient records from Beijing Tiantan Hospital, Capital Medical University, between 2013 and 2018 was performed. After identifying these 7 patients with malignant craniopharyngioma, we evaluated their demographic and clinical characteristics, including symptoms at presentation, imaging characteristics, treatment, and outcomes. RESULTS: Five patients showed transformation from benign craniopharyngiomas, and 2 had de novo malignancy. Two patients were children at diagnosis of malignancy. Patients' average age was 22 years. Headache was the most common presenting symptom, followed by visual field defects and adenohypophyseal dysfunction. Imaging characteristics showed that all patients had giant mass in the sellar, suprasellar, and/or parasellar regions that had invaded surrounding anatomic structures. Of the 5 patients who showed transformation from benign craniopharyngioma, 3 had undergone radiologic treatment before the diagnosis of malignant transformation. CONCLUSIONS:Malignant craniopharyngioma should not be overlooked in patients with a giant sellar mass, especially in young patients who have experienced multiple recurrences of craniopharyngioma and patients who received radiologic therapy.