Appalla Deepika, Depalma Andres, Calderwood Stanley
Division of Pediatric Hematology and Oncology, Department of Paediatrics, The Children's Hospital at Saint Peter's University Hospital, New Brunswick, New Jersey.
University Radiology, Saint Peter's University Hospital, New Brunswick, New Jersey.
Pediatr Blood Cancer. 2016 Jul;63(7):1276-8. doi: 10.1002/pbc.25964. Epub 2016 Mar 1.
The majority of patients with subependymal giant cell astrocytoma (SEGA) have tuberous sclerosis complex (TSC). In such patients, the mammalian target of rapamycin (mTOR) inhibitor everolimus has been shown to induce responses. Isolated SEGA have been reported in patients without clinical or genetic features of TSC. The treatment of these patients with everolimus has not previously been reported. We treated a patient with a recurrent isolated SEGA with an mTOR inhibitor. The patient tolerated therapy well and had a sustained complete remission. MTOR inhibitors may be useful for the treatment of isolated SEGA. Further study is warranted.
大多数室管膜下巨细胞星形细胞瘤(SEGA)患者患有结节性硬化症(TSC)。在这类患者中,雷帕霉素哺乳动物靶点(mTOR)抑制剂依维莫司已显示出能诱导缓解反应。有报道称,在无TSC临床或遗传特征的患者中发现了孤立性SEGA。此前尚未报道过用依维莫司治疗这些患者的情况。我们用一种mTOR抑制剂治疗了一名复发性孤立性SEGA患者。该患者对治疗耐受性良好,并实现了持续完全缓解。mTOR抑制剂可能对孤立性SEGA的治疗有用。有必要进行进一步研究。