Zhao-Jian Li1, Xiao-Lei Lan1, Feng-Yun Hao2, Wei-Cheng Yao1, Ming-Yang Wang3, Xiao-Dong Chen1, Yong-Li Bo1, Jian-Peng Wang1, Hong-Di Zhang4, Peng Sun5. 1. Department of Neurosurgery, the Affiliated Hospital of Medical College, Qingdao University, Qingdao, China. 2. Department of Pathology, the Affiliated Hospital of Medical College, Qingdao University, Qingdao, China. 3. Department of Neurosurgery, the People's Hospital of Jiaonan City, Qingdao, China. 4. Physics Scientific College, Qingdao University, Qingdao, China. 5. Department of Neurosurgery, the Affiliated Hospital of Medical College, Qingdao University, Qingdao, China. Electronic address: lizhaojian0532@163.com.
Abstract
BACKGROUND: Only four cases of primary intracerebellar paragangliomas have been reported in the literature to date. Because of its rarity, primary intracerebellar paraganglioma still presents a diagnostic challenge for both radiologists and neurosurgeons, and the optimal therapeutic modality is still debatable for its hypervascularity and location. PATIENTS: We report a 16-year-old boy with pathology-proven primary intracerebellar paraganglioma who presented with dull headache, dizziness, and gait disturbance, and underwent gross total resection. Further, we review all reported cases of primary intracerebellar paraganglioma in the English literature and discuss its clinical profile, neuroradiological features, and treatment modalities. RESULTS: His symptoms improved following tumor removal without radiotherapy, and postoperative neuroimaging thirteenth months after surgery showed no recurrence. In the literature, all four patients were stable in the follow-up period including three with complete resection and one with partial resection plus adjuvant radiotherapy. CONCLUSION: Surgical resection is the treatment modality most often used for primary intracerebellar paraganglioma; radiation therapy may be used when there is residual tumor or recurrence. Angiography may help to clarify the vessel architecture for reducing intraoperative bleeding when primary intracerebellar paraganglioma is considered.
BACKGROUND: Only four cases of primary intracerebellar paragangliomas have been reported in the literature to date. Because of its rarity, primary intracerebellar paraganglioma still presents a diagnostic challenge for both radiologists and neurosurgeons, and the optimal therapeutic modality is still debatable for its hypervascularity and location. PATIENTS: We report a 16-year-old boy with pathology-proven primary intracerebellar paraganglioma who presented with dull headache, dizziness, and gait disturbance, and underwent gross total resection. Further, we review all reported cases of primary intracerebellar paraganglioma in the English literature and discuss its clinical profile, neuroradiological features, and treatment modalities. RESULTS: His symptoms improved following tumor removal without radiotherapy, and postoperative neuroimaging thirteenth months after surgery showed no recurrence. In the literature, all four patients were stable in the follow-up period including three with complete resection and one with partial resection plus adjuvant radiotherapy. CONCLUSION: Surgical resection is the treatment modality most often used for primary intracerebellar paraganglioma; radiation therapy may be used when there is residual tumor or recurrence. Angiography may help to clarify the vessel architecture for reducing intraoperative bleeding when primary intracerebellar paraganglioma is considered.
Authors: Claudio Vernieri; Daniela Femia; Sara Pusceddu; Carlo Capella; Juan Rosai; Giuseppina Calareso; Laura Concas; Natalie Prinzi; Giuseppe Lo Russo; Filippo de Braud; Roberto Buzzoni Journal: Case Rep Oncol Date: 2016-08-17