Park Hae Gi, Yoon Jong Hyung, Kim Se Hoon, Cho Kwan Ho, Park Hyeon Jin, Kim Sun Ho, Kim Eui Hyun
Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.
Center for Pediatric Oncology, National Cancer Center, Goyang, Korea.
Brain Tumor Res Treat. 2014 Oct;2(2):108-13. doi: 10.14791/btrt.2014.2.2.108. Epub 2014 Oct 31.
We report a very rare case of sellar and suprasellar atypical teratoid rhabdoid tumor (ATRT) in a 42-year-old female patient. The tumor was removed subtotally with a transsphenoidal approach. Histopathologic study showed rhabdoid cells with prominent nucleoli and abundant cytoplasm. Immunohistochemistry for INI1 was completely negative in the tumor cells, consistent with ATRT. After surgery, she received radiotherapy including spinal irradiation with proton beam therapy and subsequent chemotherapy, with no evidence of recurrence for more than 2 years. Up to date, this is the 8th case of an adult-onset ATRT in the sellar or suprasellar region. Despite its rarity, ATRTs should be considered in the differential diagnosis of an unclear malignant sellar or suprasellar lesion in adult patients and the treatment strategies for adult ATRT patients could be differentiated from those of pediatric ATRT patients.
我们报告了一例极为罕见的鞍区和鞍上区非典型畸胎样横纹肌样瘤(ATRT),患者为一名42岁女性。采用经蝶窦入路对肿瘤进行了次全切除。组织病理学研究显示横纹肌样细胞,核仁明显,胞质丰富。肿瘤细胞中INI1免疫组化完全阴性,符合ATRT。术后,她接受了包括质子束治疗的脊髓照射及后续化疗,超过2年无复发迹象。截至目前,这是鞍区或鞍上区成人发病ATRT的第8例。尽管罕见,但在成年患者鞍区或鞍上区不明恶性病变的鉴别诊断中应考虑ATRT,且成人ATRT患者的治疗策略可与儿童ATRT患者有所不同。