Division of Hematology & Oncology, Naef K. Basile Cancer Institute, American University of Beirut Medical Center, Cairo Street, Beirut P.O. Box 11-0236, Lebanon.
Faculty of Medicine, American University of Beirut, Bliss Street, Beirut P.O. Box 11-0236, Lebanon.
Curr Oncol. 2021 Mar 20;28(2):1274-1279. doi: 10.3390/curroncol28020121.
Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR.
松果体区乳头状肿瘤（PTPR）可见于预后不良、复发率高的成年人。治疗标准包括全切除手术加放疗，由于尚未探索出长期治疗方案，因此原发性辅助化疗没有明确的前景。10 号染色体缺失是 PTPR 的特征，基因改变在广泛的人类癌症中经常发生，可使用 mTORC1 抑制剂（如依维莫司）进行治疗。同时，尚无单独使用依维莫司治疗 PTPR 的报道。我们报告了一例 PTPR（III 级）患者的病例，其特征是存在 10 号染色体缺失的改变，单独使用依维莫司进行药物治疗，结果无症状且肿瘤消退，这是一种罕见但值得注意的现象，目前文献中尚无描述，可能改变 PTPR 患者的治疗方法。
