• Zhen-Ning Xu, Xiang-Yong Yue, Xiao-Ci Cao, Ya-Dong Liu, Bao-Shuan Fang, Wen-Hao Zhao, Chen Li, Shuai Xu, and Ming Zhang.
• Department of Radiation Oncology, Hebei General Hospital, Shijiazhuang.
• Medicine (Baltimore). 2021 May 14; 100 (19): e25778.

RationaleIntracranial yolk sac tumors (YSTs) are rare malignancies with limited treatment options and a dismal prognosis. They are usually managed with surgical resection and chemoradiotherapy.Patient ConcernsHere, we report a patient with primary YST in the pineal region who achieved long term survival. Despite undergoing treatment, he experienced several recurrences over a 15-year period.DiagnosisBrain magnetic resonance imaging (MRI) demonstrated the presence of space-occupying lesions in the pineal region and the medial tail of the left lateral ventricle. The tumors were excised, and the histological diagnosis suggested an intracranial YST.InterventionsThe patient achieved long term survival after combined modality therapy including surgery, stereotactic radiosurgery (SRS)/intensity modulated radiation therapy (IMRT), chemotherapy, and targeted therapy.OutcomesThe disease remained stable. However, the patient gave up treatment and passed away in October 2020, with a total survival of about 15 years.LessonsTo the best of our knowledge, this patient with intracranial YST had received a longer survival compared with other published reports. We summarize previously published reports of intracranial YST and discuss the importance of multidisciplinary treatment. SRS may have a role, as a focal boost to residual tumor after resection or in case of recurrence after conventional radiotherapy, in the multimodality management of intracranial YSTs.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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